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Chilosi M, Piciucchi S, Ravaglia C, Spagnolo P, Sverzellati N, Tomassetti S, Wuyts W, Poletti V. "Alveolar stem cell exhaustion, fibrosis and bronchiolar proliferation" related entities. A narrative review. Pulmonology 2025; 31:2416847. [PMID: 39277539 DOI: 10.1016/j.pulmoe.2024.05.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2024] [Revised: 05/11/2024] [Accepted: 05/27/2024] [Indexed: 09/17/2024] Open
Affiliation(s)
- M Chilosi
- Department of Medical Specialities/Pulmonology Ospedale GB Morgagni, Forlì I
| | - S Piciucchi
- Department of Radiology, Ospedale GB Morgagni, Forlì I
| | - C Ravaglia
- Department of Medical Specialities/Pulmonology Ospedale GB Morgagni, Forlì (I); DIMEC, Bologna University, Forlì Campus, Forlì I, Department
| | - P Spagnolo
- Respiratory Disease Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, Padova, Italy
| | - N Sverzellati
- Scienze Radiologiche, Department of Medicine and Surgery, University Hospital Parma, Parma, Italy
| | - S Tomassetti
- Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence, Italy
| | - W Wuyts
- Pulmonology Department, UZ Leuven, Leuven, Belgium
| | - V Poletti
- Department of Medical Specialities/Pulmonology Ospedale GB Morgagni, Forlì (I); DIMEC, Bologna University, Forlì Campus, Forlì I, Department
- Department of Respiratory Diseases & Allergy, Aarhus University, Aarhus, Denmark
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Yoshinaga T, Shoji H, Imura S, Suzuki Y, Tanaka K, Takashima S, Kobori T, Hattori M, Ikubo Y, Kasai S, Okochi Y, Tokuda H. Clinical impact of radiological pleuroparenchymal fibroelastosis-like lesions in fibrotic hypersensitivity pneumonitis and efficacy of antigen avoidance. Respir Investig 2025; 63:660-666. [PMID: 40413898 DOI: 10.1016/j.resinv.2025.05.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2025] [Revised: 04/18/2025] [Accepted: 05/15/2025] [Indexed: 05/27/2025]
Abstract
BACKGROUND Pleuroparenchymal fibroelastosis (PPFE)-like lesions are frequently observed on high-resolution computed tomography in patients with fibrotic hypersensitivity pneumonitis (f-HP). This study aimed to evaluate the association of these lesions with overall survival and disease progression in f-HP patients and to examine the efficacy of antigen avoidance. METHODS A retrospective observational study of patients with f-HP was conducted in a single center. High-resolution computed tomography was used to assess the presence of radiological PPFE-like lesions. RESULTS Radiological PPFE-like lesions were detected in 48 of the 107 patients analyzed (44.9 %). Lesions were significantly associated with death from any cause (hazard ratio, 2.32; 95 % confidence interval [CI], 1.13-4.95; p = 0.024) and disease progression (odds ratio, 2.90; 95 % CI, 1.17-7.50; p = 0.024). Antigen non-avoidance was a significant predictor of worse overall survival (hazard ratio, 2.67; 95 % CI, 1.31-5.76; p = 0.008) and disease progression (odds ratio, 3.62; 95 % CI, 1.52-8.96; p = 0.004). Overall survival was significantly better (p = 0.116) and annual decline in forced vital capacity was significantly smaller (p = 0.002) in patients with radiological PPFE-like lesions who achieved antigen avoidance than in those who did not. CONCLUSIONS Radiological PPFE-like lesions were associated with worse survival and decline in forced vital capacity in patients with f-HP. Antigen avoidance may improve survival and prevent disease progression in f-HP patients with lesions.
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Affiliation(s)
- Tadatsugu Yoshinaga
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan; Department of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo, 113-8655, Japan.
| | - Hiroki Shoji
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Shingo Imura
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Yuhei Suzuki
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Kenta Tanaka
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Sae Takashima
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Tomoko Kobori
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Mototaka Hattori
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Yumiko Ikubo
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Shogo Kasai
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Yasumi Okochi
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
| | - Hitoshi Tokuda
- Department of Respiratory Medicine, Japan Community Health Care Organization Tokyo Yamate Medical Center, 3-22-1 Hyakunin-cho, Shinjuku-ku, Tokyo, 169-0073, Japan
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Ishii H, Kinoshita Y, Hamada N, Fujita M, Kushima H. Idiopathic pleuroparenchymal fibroelastosis: diagnosis and management. Expert Rev Respir Med 2025:1-12. [PMID: 40289399 DOI: 10.1080/17476348.2025.2499651] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2025] [Revised: 03/14/2025] [Accepted: 04/25/2025] [Indexed: 04/30/2025]
Abstract
INTRODUCTION Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare progressive interstitial lung disease characterized by upper-lobe fibrosis, severe restrictive impairment, and poor prognosis. Unlike idiopathic pulmonary fibrosis, in which acute exacerbations, chronic respiratory failure, and lung cancer are the major causes of death, iPPFE primarily leads to progressive respiratory failure, often complicated by malnutrition and recurrent pneumothorax. Despite growing recognition, its pathogenesis remains unclear and no effective treatments exist. AREAS COVERED This review summarizes the epidemiological, clinical, radiological, and pathological features of iPPFE, as well as diagnostic and prognostic advancements. Key prognostic factors include weight loss, reduced forced vital capacity, hypercapnia, and lower-lobe interstitial pneumonia. Serum biomarkers (e.g. latent transforming growth factor-beta binding protein-4) are being explored for early detection and prognostic purposes. Although antifibrotic agents show limited efficacy, supportive care - pulmonary rehabilitation, nutritional management, and pneumothorax prevention - remains essential. Research on the fibroelastotic pathways may inform the development of future therapies. EXPERT OPINION IPPFE remains a challenging disease. Therefore, early diagnosis and comprehensive management of this condition are crucial. Future research should refine prognostic models and explore novel therapeutic approaches for treating fibroelastosis. Lung transplantation may be an option for select patients. Further studies are required to optimize these outcomes.
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Affiliation(s)
- Hiroshi Ishii
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Yoshiaki Kinoshita
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Naoki Hamada
- Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan
| | - Masaki Fujita
- Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan
| | - Hisako Kushima
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
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4
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Gamble JL, Müller NL, Churg A, Bilawich AM. Pleuroparenchymal Fibroelastosis: Update on CT and Histologic Findings. Radiol Cardiothorac Imaging 2025; 7:e240382. [PMID: 40079759 DOI: 10.1148/ryct.240382] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/15/2025]
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is an interstitial lung disease (ILD) characterized at CT by upper lobe-predominant pleural thickening and subpleural fibrosis and histologically by visceral pleural fibrosis and subpleural fibroelastosis. Although initially classified as a rare idiopathic interstitial pneumonia, many cases are related to known risk factors, particularly hematopoietic stem cell and lung transplant, or observed in association with other ILDs. This review summarizes the diagnostic criteria for PPFE and illustrates the CT and histologic manifestations, aiming to familiarize the radiologist with the range of findings suggestive of the diagnosis. Keywords: Conventional Radiography, CT, Pulmonary, Thorax, Lung, Pleura, Complications, Transplantation, Fibrosis © RSNA, 2025.
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Affiliation(s)
- Joel L Gamble
- Department of Radiology, Vancouver General Hospital, University of British Columbia, 899 W 12th Ave, Vancouver, BC, Canada V5Z 1L5
| | - Nestor L Müller
- Department of Radiology, Vancouver General Hospital, University of British Columbia, 899 W 12th Ave, Vancouver, BC, Canada V5Z 1L5
| | - Andrew Churg
- Department of Pathology, Vancouver General Hospital, University of British Columbia, Vancouver, Canada
| | - Ana-Maria Bilawich
- Department of Radiology, Vancouver General Hospital, University of British Columbia, 899 W 12th Ave, Vancouver, BC, Canada V5Z 1L5
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Kinoshita Y, Hirai C, Hara K, Takenaka S, Utsunomiya T, Yoshida Y, Nakashima S, Kushima H, Ishii H. Diagnostic and prognostic implications of a deep suprasternal notch in idiopathic pleuroparenchymal fibroelastosis. Respir Med 2025; 238:107986. [PMID: 39921067 DOI: 10.1016/j.rmed.2025.107986] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/17/2024] [Revised: 01/21/2025] [Accepted: 02/04/2025] [Indexed: 02/10/2025]
Abstract
BACKGROUND Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a distinctive chronic interstitial lung disease characterized by upper lobe-dominant elastofibrosis. Deepening of the suprasternal notch is a notable physical feature in patients with iPPFE. However, the anatomical explanation and clinical significance of iPPFE have not yet been studied in detail. METHODS We retrospectively examined 84 patients with iPPFE, 59 with idiopathic pulmonary fibrosis (IPF), 32 with chronic hypersensitivity pneumonitis (CHP), and 91 non-interstitial lung disease (ILD) controls. The depth of the suprasternal notch assessed on axial chest computed tomography and its association with clinical, radiological, and physiological parameters, and patient outcomes were investigated. RESULTS The depth of the suprasternal notch was anatomically correlated with the thickness of the pre-tracheal soft tissue and posterior or right deviation of the trachea in patients with iPPFE. The depth of the suprasternal notch effectively discriminated patients with iPPFE from those with IPF (sensitivity, 75 %; specificity, 86.4 %), CHP (sensitivity, 75 %; specificity, 84.4 %), and non-ILD controls (sensitivity, 75 %; specificity, 83.5 %), with a cutoff value of 9.5 mm. A log-rank test showed that patients with iPPFE with a deep suprasternal notch had significantly shorter survival than those without a deep suprasternal notch. In addition, a multivariate Cox regression analysis adjusted for age, sex, and %forced vital capacity showed that the depth of the suprasternal notch was an independent risk factor for mortality. CONCLUSION The suprasternal notch is a simple and useful indicator with diagnostic and prognostic implications for patients with iPPFE.
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MESH Headings
- Humans
- Male
- Female
- Retrospective Studies
- Prognosis
- Aged
- Idiopathic Pulmonary Fibrosis/diagnostic imaging
- Idiopathic Pulmonary Fibrosis/pathology
- Idiopathic Pulmonary Fibrosis/mortality
- Idiopathic Pulmonary Fibrosis/physiopathology
- Idiopathic Pulmonary Fibrosis/diagnosis
- Tomography, X-Ray Computed/methods
- Middle Aged
- Lung Diseases, Interstitial/diagnostic imaging
- Lung Diseases, Interstitial/pathology
- Lung Diseases, Interstitial/mortality
- Lung Diseases, Interstitial/physiopathology
- Lung Diseases, Interstitial/diagnosis
- Alveolitis, Extrinsic Allergic/diagnostic imaging
- Alveolitis, Extrinsic Allergic/pathology
- Alveolitis, Extrinsic Allergic/physiopathology
- Alveolitis, Extrinsic Allergic/mortality
- Vital Capacity/physiology
- Lung/diagnostic imaging
- Lung/pathology
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Affiliation(s)
- Yoshiaki Kinoshita
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Chiharu Hirai
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Keita Hara
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Shota Takenaka
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Takuhide Utsunomiya
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Yuji Yoshida
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Shota Nakashima
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Hisako Kushima
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan
| | - Hiroshi Ishii
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan.
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Suzuki Y. Idiopathic pleuroparenchymal fibroelastosis: A review of the previous literature and current knowledge. Respir Investig 2025; 63:127-137. [PMID: 39693846 DOI: 10.1016/j.resinv.2024.12.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2024] [Revised: 11/05/2024] [Accepted: 12/06/2024] [Indexed: 12/20/2024]
Abstract
Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is characterized by upper lobe-dominant fibrosis involving the pleura and subpleural lung parenchyma. Pathologically, it is characterized by parenchymal intra-alveolar fibrosis with marked deposition of elastic fibers and dense thickening of the visceral pleura. Since iPPFE was categorized as a rare idiopathic interstitial pneumonia (IIP) by the America Thoracic Society/European Respiratory Society, several studies have been conducted, revealing an overall picture of iPPFE in terms of epidemiology, clinical manifestations, and mortality, in addition to its radiological and histological characteristics. Subsequently, several clinical diagnostic criteria that were not necessary for pathological analyses were proposed. Further, the underlying diseases responsible for secondary PPFE and PPFE-like lesions and their clinical implications were delineated. Typically, patients with iPPFE exhibit lean body stature together with platythorax, as well as relatively severe impairment of pulmonary function. In addition to upper-lobe PPFE lesions, lower-lobe interstitial lung disease (ILD) is commonly observed in patients with iPPFE, with the usual interstitial pneumonia pattern being most frequent. These distinct features of iPPFE were mostly associated with mortality, resulting in a poor prognosis relative to fibrotic ILD. Despite increased knowledge regarding the clinical characteristics of iPPFE, no effective therapy has been established other than lung transplantation. The efficacy of antifibrotic therapy, nutrition intervention, and pulmonary rehabilitation has not been determined. This article reviews previous studies and discusses the etiology, clinical manifestations, mortality risk, and treatment of iPPFE.
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Affiliation(s)
- Yuzo Suzuki
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.
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7
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Tanaka H, Asakura T, Okamori S, Furuuchi K, Yagi M, Nakayama Y, Kuramoto J, Yagi K, Hase I, Kamata H, Fujiwara K, Nakao A, Masugi Y, Sato Y, Kanai Y, Namkoong H, Fukunaga K, Nakagawa T, Morimoto K, Fujita M, Hasegawa N. Distinctive clinical features of radiological pleuroparenchymal fibroelastosis with nontuberculous mycobacterial pulmonary disease: A multicenter retrospective cohort study. Int J Infect Dis 2024; 148:107233. [PMID: 39241955 DOI: 10.1016/j.ijid.2024.107233] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/09/2024] [Revised: 08/17/2024] [Accepted: 08/30/2024] [Indexed: 09/09/2024] Open
Abstract
OBJECTIVES To compare the characteristics and prognosis of patients with nontuberculous mycobacterial (NTM) pulmonary disease (PD) with pleuroparenchymal fibroelastosis (PPFE) with those of patients with nodular/bronchiectatic (NB) and fibrocavitary (FC) NTM-PD. METHODS This multicenter, retrospective, observational study enrolled 32 patients with NTM-PPFE (median age: 70.5 years, 15 females) from six institutions in Japan from January 2003 to December 2018. Their clinical characteristics and response to therapy were compared with age- and sex-matched cohorts of patients with noncavitary NB and cavitary NB/FC NTM-PD. RESULTS Patients with NTM-PPFE had a lower body mass index and a higher standard NTM-PD therapy initiation rate than patients with other NTM-PD types. Sputum culture conversion rates were comparable between groups; however, patients with NTM-PPFE had a higher incidence of treatment-related adverse events, including optic neuropathy associated with high-dose ethambutol therapy, lower percent predicted forced vital capacity values, higher serum Krebs von den Lungen-6 (KL-6) levels, and poorer treatment outcomes than the other groups. Cox regression revealed that NTM-PPFE was an independent risk factor for death/pneumothorax (adjusted hazard ratio: 35.3, 95% confidence interval: 3.90-4692). CONCLUSION NTM-PPFE is a unique NTM-PD phenotype with a poorer prognosis than the NB and FC phenotypes.
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Affiliation(s)
- Hiromu Tanaka
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Takanori Asakura
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan; Department of Clinical Medicine (Laboratory of Bioregulatory Medicine), Kitasato University School of Pharmacy, Tokyo, Japan; Department of Respiratory Medicine, Kitasato University, Kitasato Institute Hospital, Tokyo, Japan.
| | - Satoshi Okamori
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Koji Furuuchi
- Respiratory Disease Center, Fukujuji Hospital, Japan Anti-Tuberculosis Association, Tokyo, Japan
| | - Mitsuaki Yagi
- Department of Respiratory Medicine, NHO Higashinagoya National Hospital, Nagoya, Japan
| | - Yuji Nakayama
- Department of Pathology, Keio University School of Medicine, Tokyo, Japan
| | - Junko Kuramoto
- Department of Pathology, Keio University School of Medicine, Tokyo, Japan
| | - Kazuma Yagi
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan; Department of Respiratory Medicine, NHO Tokyo Medical Center, Tokyo, Japan
| | - Isano Hase
- Department of Pulmonary Medicine, Saitama City Hospital, Saitama, Japan
| | - Hirofumi Kamata
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Keiji Fujiwara
- Respiratory Disease Center, Fukujuji Hospital, Japan Anti-Tuberculosis Association, Tokyo, Japan
| | - Akira Nakao
- Department of Respiratory Medicine, Faculty of Medicine, Fukuoka University, Fukuoka, Japan
| | - Yohei Masugi
- Division of Diagnostic Pathology, Keio University School of Medicine, Tokyo, Japan
| | - Yasunori Sato
- Department of Biostatistics, Keio University School of Medicine, Tokyo, Japan
| | - Yae Kanai
- Department of Pathology, Keio University School of Medicine, Tokyo, Japan
| | - Ho Namkoong
- Department of Infectious Diseases, Keio University School of Medicine, Tokyo, Japan
| | - Koichi Fukunaga
- Department of Medicine, Division of Pulmonary Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Taku Nakagawa
- Department of Respiratory Medicine, NHO Higashinagoya National Hospital, Nagoya, Japan
| | - Kozo Morimoto
- Respiratory Disease Center, Fukujuji Hospital, Japan Anti-Tuberculosis Association, Tokyo, Japan
| | - Masaki Fujita
- Department of Respiratory Medicine, Faculty of Medicine, Fukuoka University, Fukuoka, Japan
| | - Naoki Hasegawa
- Department of Infectious Diseases, Keio University School of Medicine, Tokyo, Japan
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Toussie D, Finkelstein M, Mendoza D, Concepcion J, Stojanovska J, Azour L, Ko JP, Moore WH, Singh A, Sasson A, Bhattacharji P, Eber C. Incidental Apical Pleuroparenchymal Scarring on Computed Tomography: Diagnostic Yield, Progression, Morphologic Features and Clinical Significance. J Thorac Imaging 2024; 39:376-384. [PMID: 38798201 DOI: 10.1097/rti.0000000000000794] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/29/2024]
Abstract
PURPOSE Apical pleuroparenchymal scarring (APPS) is commonly seen on chest computed tomography (CT), though the imaging and clinical features, to the best of our knowledge, have never been studied. The purpose was to understand APPS's typical morphologic appearance and associated clinical features. PATIENTS AND METHODS A random generator selected 1000 adult patients from all 21516 chest CTs performed at urban outpatient centers from January 1, 2016 to December 31, 2016. Patients with obscuring apical diseases were excluded to eliminate confounding factors. After exclusions, 780 patients (median age: 64 y; interquartile range: 56 to 72 y; 55% males) were included for analysis. Two radiologists evaluated the lung apices of each CT for the extent of abnormality in the axial plane (mild: <5 mm, moderate: 5 to 10 mm, severe: >10 mm), craniocaudal plane (extension halfway to the aortic arch, more than halfway, vs below the arch), the predominant pattern (nodular vs reticular and symmetry), and progression. Cohen kappa coefficient was used to assess radiologists' agreement in scoring. Ordinal logistic regression was used to determine associations of clinical and imaging variables with APPS. RESULTS APPS was present on 65% (507/780) of chest CTs (54% mild axial; 80% mild craniocaudal). The predominant pattern was nodular and symmetric. Greater age, female sex, lower body mass index, greater height, and white race were associated with more extensive APPS. APPS was not found to be associated with lung cancer in this cohort. CONCLUSION Classifying APPS by the extent of disease in the axial or craniocaudal planes, in addition to the predominant pattern, enabled statistically significant associations to be determined, which may aid in understanding the pathophysiology of apical scarring and potential associated risks.
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Affiliation(s)
- Danielle Toussie
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | - Mark Finkelstein
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | - Dexter Mendoza
- Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, Mount Sinai Hospital, New York, NY
| | - Jose Concepcion
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | | | - Lea Azour
- Department of Radiology, David Geffen School of Medicine, UCLA Medical Center, Los Angeles, CA
| | - Jane P Ko
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | - William H Moore
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | - Ayushi Singh
- Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, Mount Sinai Hospital, New York, NY
| | - Arielle Sasson
- Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, Mount Sinai Hospital, New York, NY
| | - Priya Bhattacharji
- Department of Radiology, NYU Langone Health/NYU Grossman School of Medicine
| | - Corey Eber
- Department of Diagnostic, Molecular and Interventional Radiology, Icahn School of Medicine at Mount Sinai, Mount Sinai Hospital, New York, NY
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Kondoh Y, Furukawa T, Hozumi H, Suda T, Egashira R, Jokoh T, Fukuoka J, Kuwana M, Teramachi R, Fujisawa T, Hasegawa Y, Ogura T, Miyazaki Y, Oyama S, Teramukai S, Horiguchi G, Naito A, Inoue Y, Ichikado K, Bando M, Tomioka H, Nishioka Y, Chiba H, Ebina M, Nakanishi Y, Satoh K, Shiratori Y, Hashimoto N, Ishii M. The providing multidisciplinary ILD diagnoses (PROMISE) study - study design of the national registry of Japan facilitating interactive online multidisciplinary discussion diagnosis. BMC Pulm Med 2024; 24:511. [PMID: 39396941 PMCID: PMC11472475 DOI: 10.1186/s12890-024-03232-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/06/2024] [Accepted: 08/19/2024] [Indexed: 10/15/2024] Open
Abstract
BACKGROUND Multidisciplinary discussion (MDD), in which physicians, radiologists, and pathologists communicate and diagnose together, has been reported to improve diagnostic accuracy compared to diagnoses made solely by physicians. However, even among experts, diagnostic concordance of MDD is not always good, and some patients may not receive a specific diagnosis due to insufficient findings. A provisional diagnosis based on the ontology with a diagnostic confidence level has recently been proposed. Additionally, we developed an artificial intelligence model to differentiate idiopathic pulmonary fibrosis (IPF) from other chronic interstitial lung diseases (ILD)s, which needs validation in a broader population. METHODS This prospective nationwide ILD registry has recruited patients with newly diagnosed ILD at the referral respiratory hospitals in Japan and provides rapid MDD diagnoses and treatment recommendations through a central online MDD platform with a 3-year follow-up period. A modified diagnostic ontology is used. If no diagnosis reaches more than 50% certainty, the diagnosis is unclassifiable ILD. If multiple diseases are expected, the diagnosis with a high probability takes precedence. If the confidence levels for the top two possible diagnoses are equal, the diagnosis can be unclassifiable. The registry uses tentative diagnostic criteria for nonspecific interstitial pneumonia with organising pneumonia and smoking-related ILD not otherwise specified as possible new entities. Central MDD diagnosticians review the clinical data, test results, radiology images, and pathological specimens on a dedicated website and conduct MDD diagnoses using online meetings with a cloud-based reporting system. This study aims to (1) provide MDD diagnoses with treatment recommendations; (2) determine the overall ILD rates in Japan; (3) clarify the reasons for unclassifiable ILDs; (4) evaluate possible new disease entities; (5) identify progressive phenotypes and create a clinical prediction model; (6) measure the agreement rate between institutional and central diagnoses in ILD referral and non-referral centres; (7) identify key factors for each specific ILD diagnosis; and (8) create a new disease classification system based on treatment strategies, including the use of antifibrotic drugs. DISCUSSION This study will provide ILD frequencies, including new entities, using central MDD on dedicated online systems, and develop a machine learning model for ILD diagnosis and prognosis prediction. TRIAL REGISTRATION UMIN-CTR Clinical Trial Registry (UMIN000040678).
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Affiliation(s)
- Yasuhiro Kondoh
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, 489-8642, Japan.
| | - Taiki Furukawa
- Medical IT center, Nagoya University Hospital, Nagoya, Japan
| | - Hironao Hozumi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Takafumi Suda
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Ryoko Egashira
- Department of Radiology, Faculty of Medicine, Saga University, Saga, Japan
| | - Takeshi Jokoh
- Department of Radiology, Kansai Rosai Hospital, Amagasaki, Japan
| | - Junya Fukuoka
- Department of Pathology Informatics, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
| | - Masataka Kuwana
- Department of Allergy and Rheumatology, Nippon Medical School, Tokyo, Japan
| | - Ryo Teramachi
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Tomoyuki Fujisawa
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | | | - Takashi Ogura
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Yasunari Miyazaki
- Department of Respiratory Medicine, Tokyo Medical and Dental University, Tokyo, Japan
| | - Shintaro Oyama
- Medical IT center, Nagoya University Hospital, Nagoya, Japan
| | - Satoshi Teramukai
- Department of Biostatistics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Go Horiguchi
- Department of Biostatistics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Akari Naito
- The Clinical and Translational Research Center, University Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
| | - Yoshikazu Inoue
- Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan
| | - Kazuya Ichikado
- Division of Respiratory Medicine, Saiseikai Kumamoto Hospital, Kumamoto, Japan
| | - Masashi Bando
- Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Tochigi, Japan
| | - Hiromi Tomioka
- Department of Respiratory Medicine, Kobe City Medical Center West Hospital, Kobe, Japan
| | - Yasuhiko Nishioka
- Department of Respiratory Medicine and Rheumatology, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan
| | - Hirofumi Chiba
- Department of Respiratory Medicine and Allergology, Sapporo Medical University School of Medicine, Sapporo, Japan
| | - Masahito Ebina
- Department of Respiratory Medicine, Tohoku Medical and Pharmaceutical University School of Medicine, Sendai, Japan
| | | | - Kikue Satoh
- Medical IT center, Nagoya University Hospital, Nagoya, Japan
| | | | - Naozumi Hashimoto
- Department of Respiratory Medicine, Fujita Health University, Toyoake, Japan
| | - Makoto Ishii
- Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Japan
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10
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Yamakawa H, Oda T, Sugino K, Hirama T, Komatsu M, Katano T, Fukuda T, Takemura T, Kubota Y, Kishaba T, Norisue Y, Araya J, Ogura T. Proposed Clinical Algorithm for Pleuroparenchymal Fibroelastosis (PPFE). J Clin Med 2024; 13:3675. [PMID: 38999241 PMCID: PMC11242275 DOI: 10.3390/jcm13133675] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/17/2024] [Revised: 06/19/2024] [Accepted: 06/21/2024] [Indexed: 07/14/2024] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is characterized by fibrosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobes. As PPFE appears to occur in patients with heterogeneous etiologies, the disease course is thus also heterogenous, with some patients showing rapid progression while others have slow progression. Therefore, it is very difficult to predict prognosis with PPFE. Needless to say, this problematic matter has influenced the treatment strategy of PPFE patients. In fact, until now no evidence has been shown for use in creating an appropriate management algorithm for PPFE. We speculate that "uncoordinated breathing" is the most important reason for dyspnea in PPFE patients. Because monitoring of physique and not just pulmonary function and radiological evaluation is also very important, particularly in PPFE patients, this review focused on the characteristics of PPFE through an overview of previous studies in this field, and we proposed an algorithm as precision medicine based on the current evidence. Multiple views by the pulmonologist are needed to standardize a clinical algorithm that is necessary to correctly assess PPFE patients under the premise of maintenance of physique by providing appropriate nutritional care and pulmonary rehabilitation.
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Affiliation(s)
- Hideaki Yamakawa
- Department of Respiratory Medicine, Saitama Red Cross Hospital, Saitama 330-8553, Japan
- Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo 105-8461, Japan
| | - Tsuneyuki Oda
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan
| | - Keishi Sugino
- Department of Respiratory Medicine, Tsuboi Hospital, Fukushima 963-0197, Japan
| | - Takashi Hirama
- Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai 980-8575, Japan
- Division of Organ Transplantation, Tohoku University, Sendai 980-8574, Japan
| | - Masamichi Komatsu
- First Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto 390-8621, Japan
| | - Takuma Katano
- Department of Respiratory Medicine and Allergology, Aichi Medical University, Nagakute 480-1195, Japan
| | - Taiki Fukuda
- Department of Radiology, The Jikei University School of Medicine, Tokyo 105-8461, Japan
| | - Tamiko Takemura
- Department of Pathology, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan
| | - Yoshiaki Kubota
- Department of Cardiovascular Medicine, Nippon Medical School, Tokyo 113-0022, Japan
| | - Tomoo Kishaba
- Department of Respiratory Medicine, Okinawa Chubu Hospital, Okinawa 904-2293, Japan
| | - Yasuhiro Norisue
- Department of Emergency and Critical Care Medicine, Tokyo Bay Urayasu Ichikawa Medical Center, Chiba 279-0001, Japan
| | - Jun Araya
- Department of Respiratory Medicine, Tokyo Jikei University Hospital, Tokyo 105-8461, Japan
| | - Takashi Ogura
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama 236-0051, Japan
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11
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Shiraishi M, Higashimoto Y, Nishiyama O, Yamazaki R, Sugiya R, Mizusawa H, Takeda Y, Noguchi M, Kanki K, Kuwano M, Kimura T, Matsumoto H. Improvement of diaphragmatic excursion in the supine position in a patient with idiopathic pleuroparenchymal fibroelastosis: A case report. Respir Med Case Rep 2024; 51:102078. [PMID: 39027817 PMCID: PMC11255951 DOI: 10.1016/j.rmcr.2024.102078] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/26/2023] [Revised: 05/03/2024] [Accepted: 06/20/2024] [Indexed: 07/20/2024] Open
Abstract
Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a rare form of idiopathic interstitial pneumonia. We report a case of a patient with iPPFE in whom postural changes improved diaphragmatic excursion (DE) and exercise tolerance. Chest radiography showed a greater elevation of the diaphragm at maximum expiration in the supine position than the standing position. DE measured by ultrasonography was higher in the supine position than the standing position. The findings may suggest greater diaphragm movement in the supine position, leading to successful rehabilitation and improved exercise endurance. There is no effective treatment for iPPFE; therefore, an innovative treatment strategy is warranted.
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Affiliation(s)
- Masashi Shiraishi
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Yuji Higashimoto
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Osamu Nishiyama
- Department of Respiratory Medicine and Allergology, Kindai University Faculty of Medicine, Osakasayama, Japan, Osaka, Japan
| | - Ryo Yamazaki
- Department of Respiratory Medicine and Allergology, Kindai University Faculty of Medicine, Osakasayama, Japan, Osaka, Japan
| | - Ryuji Sugiya
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Hiroki Mizusawa
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Yu Takeda
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Masaya Noguchi
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Kengo Kanki
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Mitsuo Kuwano
- Department of Radiology Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Tamotsu Kimura
- Department of Rehabilitation Medicine, Kindai University Faculty of Medicine, Osaka, Japan
| | - Hisako Matsumoto
- Department of Respiratory Medicine and Allergology, Kindai University Faculty of Medicine, Osakasayama, Japan, Osaka, Japan
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12
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Kumar P, Hashmi S, Birbeck M, Seklani M, Subramanian N. A Rare Case of Idiopathic Pleuro-Parenchymal Fibroelastosis Presenting With Bilateral Spontaneous Pneumothoraces. Cureus 2024; 16:e56975. [PMID: 38665718 PMCID: PMC11045163 DOI: 10.7759/cureus.56975] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/26/2024] [Indexed: 04/28/2024] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD), characterized by predominantly upper lobe pleural and subjacent sub-pleural parenchymal fibrosis. Its name refers to a combination of fibrosis involving the visceral pleura with fibroelastotic changes, predominantly in the subpleural lung parenchyma. We describe the case of a 67-year-old lady who presented to the accident and emergency department of Weston General Hospital with worsening shortness of breath (SOB) and cachexia of six to eight months' duration. The initial imaging studies showed bilateral spontaneous pneumothoraces on a background of pleural-based consolidation and fibrotic changes. A subsequent high-resolution CT (HRCT) chest showed evidence of pleuroparenchymal fibroelastosis in the background. She was not considered for anti-fibrotic medications due to the advanced stage of the disease and was managed with supportive measures, including oxygen support, oral steroids and antibiotics to cover for any infections. After initial management of symptoms and long discussions with the patient, family and the palliative team, she was discharged home with community follow-up.
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Affiliation(s)
- Prasad Kumar
- Pulmonology, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, GBR
| | - Shahnawaz Hashmi
- Internal Medicine, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, GBR
| | - Matthew Birbeck
- Pulmonary Medicine, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, GBR
| | - Mohamed Seklani
- Pulmonary Medicine, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, GBR
| | - Nilaa Subramanian
- Internal Medicine, University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, GBR
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13
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Fujisaki M, Higuchi T, Konda N, Hirahara S, Watanabe K, Yamada R, Motoyama R, Yamaguchi R, Katsumata Y, Kawaguchi Y, Harigai M. Development of microscopic polyangiitis following idiopathic pleuroparenchymal l fibroelastosis: A case report. Mod Rheumatol Case Rep 2023; 8:141-144. [PMID: 37307430 DOI: 10.1093/mrcr/rxad035] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2023] [Revised: 05/27/2023] [Accepted: 06/08/2023] [Indexed: 06/14/2023]
Abstract
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, which is characterised by pleural fibrosis and subjacent parenchymal fibroelastosis of the upper lobes. Herein, we present a case of microscopic polyangiitis (MPA) following PPFE. The patient had abnormal shadows on chest radiographs 15 years before the onset of MPA, and the patient was diagnosed with PPFE. Four years after the PPFE diagnosis, the patient was diagnosed with MPA based on persistent fever, purpura, mononeuritis multiplex, myeloperoxidase-antineutrophil cytoplasmic antibody positivity, and pathological findings of peritubular capillaritis on kidney biopsy. The patient was treated with glucocorticoids, including methylprednisolone pulse therapy and rituximab, followed by maintenance therapy with rituximab. One year after treatment, the PPFE had not worsened. PPFE occasionally occurs secondary to connective tissue disease, including MPA; however, to the best of our knowledge, this is the first report of PPFE preceding MPA. Our case suggests that PPFE, as other interstitial lung diseases, may be associated with MPA and precede the onset of MPA. The accumulation of more cases is needed to clarify the characteristics of MPA-associated PPFE.
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Affiliation(s)
- Mayuko Fujisaki
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
- Department of Rheumatology, Tokyo Metropolitan Ohtsuka Hospital, Tokyo, Japan
| | - Tomoaki Higuchi
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
- Division of Multidisciplinary Management of Rheumatic Diseases, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Naoko Konda
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Shinya Hirahara
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Kotaro Watanabe
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Risa Yamada
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Ryo Motoyama
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Rei Yamaguchi
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Yasuhiro Katsumata
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Yasushi Kawaguchi
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
| | - Masayoshi Harigai
- Division of Rheumatology, Department of Internal Medicine, Tokyo Women's Medical University School of Medicine, Tokyo, Japan
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14
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Yombo DJK, Madala SK, Vemulapalli CP, Ediga HH, Hardie WD. Pulmonary fibroelastosis - A review. Matrix Biol 2023; 124:1-7. [PMID: 37922998 PMCID: PMC10841596 DOI: 10.1016/j.matbio.2023.10.003] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2023] [Revised: 10/11/2023] [Accepted: 10/31/2023] [Indexed: 11/07/2023]
Abstract
Elastin is a long-lived fibrous protein that is abundant in the extracellular matrix of the lung. Elastic fibers provide the lung the characteristic elasticity during inhalation with recoil during exhalation thereby ensuring efficient gas exchange. Excessive deposition of elastin and other extracellular matrix proteins reduces lung compliance by impairing ventilation and compromising gas exchange. Notably, the degree of elastosis is associated with the progressive decline in lung function and survival in patients with interstitial lung diseases. Currently there are no proven therapies which effectively reduce the elastin burden in the lung nor prevent dysregulated elastosis. This review describes elastin's role in the healthy lung, summarizes elastosis in pulmonary diseases, and evaluates the current understanding of elastin regulation and dysregulation with the goal of guiding future research efforts to develop novel and effective therapies.
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Affiliation(s)
- Dan J K Yombo
- Division of Pulmonary Medicine, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine Cincinnati, OH, USA
| | - Satish K Madala
- Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio USA
| | - Chanukya P Vemulapalli
- Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio USA
| | - Harshavardhana H Ediga
- Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio USA
| | - William D Hardie
- Division of Pulmonary Medicine, Department of Pediatrics, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine Cincinnati, OH, USA.
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15
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Piciucchi S, Fernades LS, Ravaglia C, Tomassetti S, Garo ML, Poletti V. "Gallia est omnis divisa in partes tres": Is it time to divide Pleuroparenchymal Fibroelastosis in three different forms? Pulmonology 2023; 29:550-554. [PMID: 37210339 DOI: 10.1016/j.pulmoe.2023.04.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2023] [Revised: 03/23/2023] [Accepted: 04/12/2023] [Indexed: 05/22/2023] Open
Affiliation(s)
- Sara Piciucchi
- Department of Radiology, GB Morgagni Pierantoni Hospital/University of Bologna, Italy.
| | | | - Claudia Ravaglia
- Pulmonology Unit, G.B. Morgagni Hospital/University of Bologna, Forlì, Italy
| | - Sara Tomassetti
- Department of Experimental & Clinical Medicine, University of Florence, Florence, Italy
| | - Maria Luisa Garo
- Università Campus Bio-medico di Roma, UOC di Cardiochirurgia, Rome, Italy
| | - Venerino Poletti
- Alma Mater Studiorum University of Bologna, DIMEC, Bologna, Italy; Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark
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16
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Gentili F, Di Martino V, Forestieri M, Mazzei F, Guerrini S, Bargagli E, Sisinni AG, Volterrani L, Mazzei MA. Pleuroparenchymal Fibroelastosis-like Lesions in Clinical Practice: A Rare Entity? Review of a Radiological Database. Diagnostics (Basel) 2023; 13:diagnostics13091627. [PMID: 37175018 PMCID: PMC10178180 DOI: 10.3390/diagnostics13091627] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2023] [Revised: 03/29/2023] [Accepted: 04/04/2023] [Indexed: 05/15/2023] Open
Abstract
Background: Pleuroparenchymal Fibroelastosis (PPFE) is a rare disease that consists of elastofibrosis that involves the pleura and subpleural lung parenchyma; it is an unusual pulmonary disease with unique clinical, radiological and pathological characteristics. According to recent studies, PPFE may not be a definite disease but a form of chronic lung injury. The aim of this retrospective study is to determine the incidence and to evaluate the distribution, severity and progression of this radiological entity on high-resolution CT (HRCT) exams of the chest, performed in routine clinical practice. In total, 1514 HRCT exams performed in the period January 2016-June 2018 were analyzed. For each exam, the presence of PPFE was evaluated and a quantitative score was assigned (from 0 to 7 points, based on the maximum depth of fibrotic involvement of the parenchyma). When available, two exams with a time interval of at least 6 months were compared for each patient in order to evaluate progression (defined as the increase in the disease score). Patients were divided into different groups according to exposure and their associated diseases. Statistical analysis was performed by using the Wilcoxon test and Kruskal-Wallis test. Results: PPFE was detected in 174 out of 1514 patients (11.6%), with a mean score of 6.1 ± 3.9 (range 1-14). In 106 out of 174 patients (60.9%), a previous CT scan was available and an evolution of PPFE was detected in 19 of these (11.5%). Among these 19 patients with worsening PPFE, 4 had isolated PPFE that was associated with chronic exposure or connective tissue disorders, and the other 15 had an associated lung disease and/or a chronic exposure. In this group, it was found that the ventral segments of the upper lobes, fissures and apical segments of the lower lobes had a greater statistically significant involvement in the progression of the disease compared to the non-progressive group. In 16 of 174 patients (9.2%, 7 of which belonged to the radiological progression group) a biopsy through video-assisted thoracoscopic surgery or apicoectomy confirmed PPFE. Conclusion: PPFE-like lesions are not uncommon on HRCT exams in routine clinical practice, and are frequently found in patients with different forms of chronic lung injury. Further studies are necessary to explain why the disease progresses in some cases, while in most, it remains stationary over time.
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Affiliation(s)
- Francesco Gentili
- Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
| | - Vito Di Martino
- Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
| | | | - Francesco Mazzei
- Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
| | - Susanna Guerrini
- Diagnostic Imaging, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
| | - Elena Bargagli
- Respiratory Diseases and Lung Transplant Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, 53100 Siena, Italy
| | | | - Luca Volterrani
- Department of Radiological Sciences, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
| | - Maria Antonietta Mazzei
- Department of Radiological Sciences, Azienda Ospedaliera Universitaria Senese, 53100 Siena, Italy
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17
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Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R. The world of rare interstitial lung diseases. Eur Respir Rev 2023; 32:32/167/220161. [PMID: 36754433 PMCID: PMC9910344 DOI: 10.1183/16000617.0161-2022] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2022] [Accepted: 12/21/2022] [Indexed: 02/10/2023] Open
Abstract
The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and therapy usually pose challenges. This review describes a selection of rare and ultrarare ILDs including pulmonary alveolar proteinosis, pulmonary alveolar microlithiasis and pleuroparenchymal fibroelastosis. In addition, monogenic ILDs or ILDs in congenital syndromes and various multiple cystic lung diseases will be discussed. All these conditions are part of the scope of the European Reference Network on rare respiratory diseases (ERN-LUNG). Epidemiology, pathogenesis, diagnostics and treatment of each disease are presented.
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Affiliation(s)
- Katharina Buschulte
- Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), ERN-LUNG, Heidelberg, Germany
| | - Vincent Cottin
- National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, ERN-LUNG, Lyon, France
| | - Marlies Wijsenbeek
- Center for Interstitial Lung Diseases and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC-University Medical Center, ERN-LUNG, Rotterdam, The Netherlands
| | - Michael Kreuter
- Center for Interstitial and Rare Lung Diseases, Thoraxklinik, University of Heidelberg, German Center for Lung Research (DZL), ERN-LUNG, Heidelberg, Germany
| | - Rémi Diesler
- National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, ERN-LUNG, Lyon, France
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18
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Clinical features of idiopathic pleuroparenchymal fibroelastosis with progressive phenotype showing a decline in forced vital capacity. Respir Investig 2023; 61:210-219. [PMID: 36773509 DOI: 10.1016/j.resinv.2023.01.003] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/14/2022] [Revised: 12/12/2022] [Accepted: 01/06/2023] [Indexed: 02/11/2023]
Abstract
BACKGROUND Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is heterogeneous, with some patients showing a progressive decline in forced vital capacity (FVC). However, the clinical features of these cases with progressive phenotypes remain unknown. METHODS This retrospective study included 48 patients diagnosed with IPPFE who underwent longitudinal pulmonary function tests at our institution from 2005 to 2021. The progressive phenotype was defined as a relative decline of ≥10% in %FVC within two years from diagnosis of IPPFE, and its clinical features were evaluated. RESULTS Of the 48 patients, 23 (47.9%) were classified as progressive IPPFE. They were significantly older with a higher rate of dyspnea, fine crackles on chest auscultation, lower-lobe usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography, and lower %FVC at diagnosis than non-progressive IPPFE. Additionally, progressive IPPFE had a significantly higher rate of long-term oxygen therapy requirement, the incidence of pneumothorax, and weight loss after diagnosis, which showed worse survival than non-progressive IPPFE. The relative decline in %FVC and weight loss showed a significant positive correlation. Multivariate analysis revealed that lower body mass index tended to predict early progression, and the coexistence of lower-lobe UIP pattern was significantly associated with early progression. A decline in %FVC was an independent poor prognostic factor in IPPFE. CONCLUSIONS With a progressive decline in %FVC, IPPFE often has an advanced stage at diagnosis and lower-lobe UIP pattern and is associated with weight loss and worse survival.
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19
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Matsumura T, Makino T, Yagisawa M, Ishige M, Akimoto Y, Ito K, Anami Y, Kono M. Acute exacerbation of pleuroparenchymal fibroelastosis with lower lobe usual interstitial pneumonia: An autopsy case. Respir Med Case Rep 2023; 43:101846. [PMID: 37077237 PMCID: PMC10106554 DOI: 10.1016/j.rmcr.2023.101846] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2023] [Revised: 03/24/2023] [Accepted: 04/06/2023] [Indexed: 04/21/2023] Open
Abstract
An 87-year-old man presented with dyspnea. Computed tomography revealed progressive subpleural consolidation in the apex, reticular shadows in the lower lobes, and bilateral ground glass opacifications. He died of respiratory failure on day 3. The post-mortem examination showed exudative stage diffuse alveolar damage and pulmonary edema. Intraalveolar collagenous fibrosis and subpleural elastosis were observed in the upper lobes, accompanied by interlobular septal and pleural thickening and lung architecture remodeling in the lower lobes. He was diagnosed with acute exacerbation of pleuroparenchymal fibroelastosis with lower lobe usual interstitial pneumonia, which can be fatal.
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Affiliation(s)
- Takuma Matsumura
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
- Corresponding author.
| | - Takashi Makino
- Department of Respiratory Surgery, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Marie Yagisawa
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Masaki Ishige
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Yuto Akimoto
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Kosuke Ito
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Yoichi Anami
- Department of Respiratory Surgery, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
| | - Masakazu Kono
- Department of Respiratory Medicine, Tokyo Rosai Hospital, 4-13-21, Ohmori-Minami, Ohta-ku, Tokyo, Japan
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20
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Franquet T, Giménez Palleiro A. Idiopathic pleuroparenchymal fibroelastosis. RADIOLOGIA 2022; 64 Suppl 3:301-307. [PMID: 36737168 DOI: 10.1016/j.rxeng.2022.12.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2022] [Accepted: 10/12/2022] [Indexed: 02/05/2023]
Abstract
The term idiopathic pleuroparenchymal fibroelastosis refers to a rare interstitial lung disease that predominantly involves the upper lobes. It has been considered a rare subtype of interstitial lung disease since 2013, when it was included in the joint consensus statement on the diagnosis of interstitial lung diseases published by the American Thoracic Society (ATS) and the European Respiratory Society (ERS). Currently, two distinct types of pleuroparenchymal fibroelastosis are recognized: the idiopathic type for cases in which it has not been possible to establish a specific etiology and a secondary type associated with a variety of different causes. The diagnosis of pleuroparenchymal fibroelastosis must be managed from a combined clinical and radiological perspective. High-resolution computed tomography (HRCT) is the imaging method of choice for the evaluation and diagnosis of pleuroparenchymal fibroelastosis. In many cases, the diagnosis will be based exclusively on the HRCT findings and histologic confirmation will be unnecessary. This article describes the clinical, radiological, and histological characteristics of pleuroparenchymal fibroelastosis, discussing the different associations with this entity and its differential diagnosis.
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Affiliation(s)
- T Franquet
- Servicio de Radiodiagnóstico, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
| | - A Giménez Palleiro
- Servicio de Radiodiagnóstico, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
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Aono Y, Hozumi H, Kono M, Hashimoto D, Nakamura H, Yokomura K, Imokawa S, Shirai M, Akahori D, Inoue Y, Mori K, Karayama M, Suzuki Y, Furuhashi K, Enomoto N, Fujisawa T, Nakamura Y, Inui N, Suda T. Prognostic significance of radiological pleuroparenchymal fibroelastosis in Mycobacterium aviumcomplex lung disease: a multicentre retrospective cohort study. Thorax 2022:thorax-2022-219116. [DOI: 10.1136/thorax-2022-219116] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2022] [Accepted: 10/18/2022] [Indexed: 11/13/2022]
Abstract
BackgroundMycobacterium aviumcomplex (MAC) causes chronic respiratory infectious diseases with diverse clinical features and prognoses. Pleuroparenchymal fibroelastosis (PPFE) is a rare disease characterised by pleural fibrosis with subjacent intra-alveolar fibrosis and alveolar septal elastosis, with unique chest high-resolution CT (HRCT) features (radiological PPFE). An association between recurrent respiratory infections and PPFE formation has been hypothesised; however, the clinical significance of PPFE in MAC lung disease remains unclear.MethodsThis retrospective, multicentre study investigated the prevalence of radiological PPFE in patients with MAC lung disease and its association with clinical features and outcomes. Radiological PPFE was diagnosed on the basis of HRCT findings. Prognostic factors were identified using Cox proportional hazards and Fine-Gray models.ResultsOf 850 consecutive patients with definite MAC lung disease, 101 (11.9%) exhibited radiological PPFE. Patients with radiological PPFE had unique characteristics, such as lower body mass index, lower survival rate (5-year cumulative survival rate, 63.1% vs 91.7%; p<0.001) and a higher incidence of respiratory-related death (5-year cumulative incidence, 31.1% vs 3.6%; p<0.001), than those without radiological PPFE. In the multivariable analysis, the presence of radiological PPFE was independently associated with all-cause mortality (adjusted HR, 4.78; 95% CI, 2.87 to 7.95; p<0.001) and respiratory-related death (adjusted HR, 3.88; 95% CI, 2.14 to 7.01; p<0.001).InterpretationThis large-scale study demonstrated that in patients with MAC lung disease, radiological PPFE was common, a phenotype associated with unique clinical features and poor prognosis, particularly respiratory-related death. The specific management of this subgroup should be established.
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22
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Fibroelastosis pleuroparenquimatosa idiopática (FEPPI). RADIOLOGIA 2022. [DOI: 10.1016/j.rx.2022.10.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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Tetikkurt C, Ozturk BC, Gungordu N. Diagnosis of pleuroparenchymal fibroelastosis: A review. Monaldi Arch Chest Dis 2022. [PMID: 36269206 DOI: 10.4081/monaldi.2022.2363] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2022] [Accepted: 10/10/2022] [Indexed: 06/16/2023] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare lung disease with unprecedented features characterized by fibroelastotic changes in the subpleural lung parenchyma affecting the upper lobes. PPFE is usually idiopathic, but it can be caused by infection, autoimmunity, bone marrow or lung transplantation, or a genetic predisposition. Histopathologic examination of lung biopsy samples reveals homogenous subpleural fibrosis and abundant elastic fibers, allowing for a definitive diagnosis. As PPFE mimics many interstitial lung diseases, clinicians face significant difficulties in making a definitive final diagnosis. Since most disease-related comorbid conditions manifest at an advanced stage, invasive tissue sampling for histopathologic evaluation is consistently impossible. Such a patient presentation highlights the importance of an analysis based solely on clinical findings, which would provide a definitive diagnosis without the need for a biopsy. Because of its exceptional and inconceivable presentation, PPFE creates a diagnostic dilemma. In light of our two cases and the literature data, we present a diagnostic assessment score assay that relies solely on clinical manifestations without histopathological tissue verification to shed light on the diagnosis of PPFE. This review focuses on PPFE identification through the use of a diagnostic assessment analysis to improve early disease recognition without the use of invasive diagnostic interventions to obtain biopsy samples for histopathologic evaluation. This analytic approach, while not diagnostic in and of itself, may provide a useful pathway for differential diagnosis and may preclude redundant initiatives.
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Affiliation(s)
- Cuneyt Tetikkurt
- Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University, Istanbul.
| | - Buket Caliskaner Ozturk
- Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University, Istanbul.
| | - Nejdiye Gungordu
- Department of Pulmonary Diseases, Cerrahpasa Medical Faculty, Istanbul Cerrahpasa University, Istanbul.
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Sato N, Iwanami Y, Ebihara K, Nakao K, Miyagi M, Nakamura Y, Kishi K, Homma S, Ebihara S. Determinants of Six-Minute Walk Distance in Idiopathic Pulmonary Fibrosis and Idiopathic Pleuroparenchymal Fibroelastosis. Biomedicines 2022; 10:biomedicines10102556. [PMID: 36289817 PMCID: PMC9599550 DOI: 10.3390/biomedicines10102556] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2022] [Revised: 10/03/2022] [Accepted: 10/10/2022] [Indexed: 12/01/2022] Open
Abstract
Background: In idiopathic pulmonary fibrosis (IPF), 6-minute walking distance (6MWD) is an independent factor for mortality. Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disease with physical features such as emaciation, but the relationship between IPPFE and 6MWD is unclear. In this study, we investigated the factors that cause a decrease in the percent of the predicted value of a 6-minute walk distance (%6MWD), including the disease entities, IPF and IPPFE. Methods: This study included 100 patients (IPF: 75 patients, IPPFE: 25 patients, age: 73.5 ± 7.2 years, sex: 73 males) who visited the rehabilitation department. Patients with a %6MWD ≥ 80% were assigned to the normal group (n = 54), and patients with a %6MWD < 80% were assigned to the decreased group (n = 46). The items showing a significant difference between groups were used as independent variables, after the consideration of multicollinearity, for a logistic analysis where %6MWD < 80% was used as the dependent variable. Results: The 6MWD results show that there was no significant difference between IPF and IPPFE in the absolute value of 6MWD and in the number of people with 6MWD ≥ 250 m, but when 6MWD was compared with %6MWD, the IPPFE group showed a significantly lower value than the IPF group (p = 0.013). Logistic regression analysis showed that only BMI (p = 0.032), GAP index (p = 0.043), and mMRC (p = 0.026) were factors that caused a decrease in %6MWD in 100 patients. Conclusion: The results suggest that leanness, shortness of breath and severity of illness are the most important factors that determine exercise tolerance, regardless of disease entity in IPF and IPPFE.
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Affiliation(s)
- Naofumi Sato
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Yuji Iwanami
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Kento Ebihara
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Keiko Nakao
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Midori Miyagi
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Yasuhiko Nakamura
- Department of Respiratory Medicine, Toho University School of Medicine, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Kazuma Kishi
- Department of Respiratory Medicine, Toho University School of Medicine, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Sakae Homma
- Department of Advanced and Integrated Interstitial Lung Diseases Research, Toho University School of Medicine, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
| | - Satoru Ebihara
- Department of Rehabilitation Medicine, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan
- Department of Internal Medicine and Rehabilitation Science, Tohoku University Graduate School of Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai 980-5874, Japan
- Correspondence:
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Haloui O, El Allam F, Nabou O, Thouil A, Kouismi H. Pleuroparenchymal Fibroelastosis: A Case Report. Cureus 2022; 14:e29211. [PMID: 36259005 PMCID: PMC9569183 DOI: 10.7759/cureus.29211] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/14/2022] [Indexed: 11/22/2022] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare, idiopathic interstitial pneumonia. wherein the first symptom might be dyspnea or a dry cough. The condition can also be manifested with chest pain secondary to pneumothorax. While the definitive diagnosis is based on a histological evaluation (which is not often performed), a computed tomography scan shows findings, such as apical fibrosis and pleural thickening of the apical lobes, which help assess the diagnosis. We describe a case of PPFE diagnosed radiologically in a 69-year-old man. This case highlights that PPFE is a pathology that can go unnoticed for a long time, and patients might neglect the revealing symptoms such as coughing.
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Cottin V, Si-Mohamed S, Diesler R, Bonniaud P, Valenzuela C. Pleuroparenchymal fibroelastosis. Curr Opin Pulm Med 2022; 28:432-440. [PMID: 35855575 DOI: 10.1097/mcp.0000000000000907] [Citation(s) in RCA: 19] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/24/2023]
Abstract
PURPOSE OF REVIEW Pleuroparenchymal fibroelastosis (PPFE) is a clinico-radiologic-pathologic interstitial lung disease (ILD) characterized by fibrosis that has upper lobe and subpleural predominance, involving both the visceral pleura and the subjacent subpleural lung parenchyma, and comprises dense fibroelastic changes with prominent elastosis of the alveolar walls together with fibrous thickening of the visceral pleura. The goal of this review is to summarize the state-of-the-art understanding in PPFE. RECENT FINDINGS PPFE was described in an increasing number of conditions. The course of disease is heterogeneous. Idiopathic PPFE, cases associated with telomerase-related gene mutations, cases related to a history of chemotherapy, and cases combining PPFE with a pattern of usual interstitial pneumonia, may have a particularly poor prognosis. Well-conducted retrospective studies identified marked PPFE features in approximately 10% of patients with idiopathic pulmonary fibrosis, 11% of patients with systemic sclerosis-associated ILD, 6.5% of patients with rheumatoid arthritis-associated ILD, and 23% of patients with hypersensitivity pneumonitis. Drug therapy has not been evaluated prospectively. A small retrospective study suggests that nintedanib may slow disease progression. However, whether the efficacy of antifibrotics is comparable in PPFE and in other forms of progressive pulmonary fibrosis warrants further evaluation. SUMMARY Accumulating data indicate that PPFE features are associated with poor prognosis in fibrosing ILDs. Further research on the management of PPFE is warranted.
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Affiliation(s)
- Vincent Cottin
- Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases
| | - Salim Si-Mohamed
- Department of Thoracic Imaging, Louis Pradel Hospital, Hospices Civils de Lyon, UMR 754, Claude Bernard University Lyon 1, Lyon
| | - Rémi Diesler
- Department of Respiratory Medicine, National Reference Centre for Rare Pulmonary Diseases
| | - Philippe Bonniaud
- Constitutive Reference Center for Rare Pulmonary Diseases, Department of Pulmonary Medicine and Intensive Care Unit, Inserm U1231, University of Bourgogne-Franche Comté, Dijon, France
| | - Claudia Valenzuela
- Department of Respiratory Medicine, Hospital universitario de la Princesa, Universitad autónoma de Madrid, Madrid, Spain
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Cueto-Robledo G, Guerrero-Velazquez JF, Roldan-Valadez E, Graniel-Palafox LE, Cervantes-Naranjo FD, Cueto-Romero HD, Rivera-Sotelo N. Pulmonary hypertension or pulmonary arterial hypertension in idiopathic pleuroparenchymal fibroelastosis: An updated comprehensive review. Curr Probl Cardiol 2022; 47:101368. [PMID: 36028054 DOI: 10.1016/j.cpcardiol.2022.101368] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2022] [Accepted: 08/16/2022] [Indexed: 11/03/2022]
Abstract
Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a little-known entity with unique clinical, radiological, and pathological features. iPPFE is chronic interstitial pneumonia characterized by the thickening of elastic fibers in the pleura and subpleural parenchyma involving the upper lobes. Computed tomography pulmonary angiography (CTPA) usually depicts bilateral pleural thickening, with a left scalloped appearance that conditions retraction of the structures of the superior mediastinum and both pulmonary hila, associated with pulmonary consolidations with bronchogram air and thickening of the peribronchovascular interstitium, in addition to areas of left apical air trapping. When severe enough, the disease leads to progressive loss of volume of the upper lobes, decreased body mass, and platythorax. Some patients with iPPFE follow an inexorably progressive course culminating in irreversible respiratory failure and premature death. Up to 20% of patients might develop pulmonary hypertension (PH); transthoracic echocardiography is used as a screening test for PH; right heart catheterization performed in a tertiary-care hospital will confirm the diagnosis. Because iPPFE can be easily confused and misdiagnosed with infectious pathologies, such as pulmonary tuberculosis, and easily confuse physicians with little expertise in diffuse interstitial lung diseases, knowing the differential diagnoses, clinical presentation, imaging, and complications of the iPPFE allows for an early diagnosis and gives patients who suffer from it a better quality of life. This report presents a comprehensive review of PPFEi, discussing severe precapillary pulmonary hypertension and the associated findings demonstrated by right heart catheterization (RHC), which be of interest for cardiopulmonologists.
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Affiliation(s)
- Guillermo Cueto-Robledo
- Cardiorespiratory Emergencies, Hospital General de México "Dr Eduardo Liceaga", 06720, Mexico City, Mexico; Pulmonary Circulation Clinic, Hospital General de México "Dr. Eduardo Liceaga", 06720, Mexico City, Mexico; Faculty of Medicine, National Autonomous University of Mexico, Mexico City, Mexico.
| | | | - Ernesto Roldan-Valadez
- Directorate of Research, Hospital General de Mexico "Dr. Eduardo Liceaga," 06720, Mexico City, Mexico; I.M. Sechenov First Moscow State Medical University (Sechenov University), Department of Radiology, 119992, Moscow, Russia.
| | | | | | - Hector-Daniel Cueto-Romero
- Cardiorespiratory Emergencies, Hospital General de México "Dr Eduardo Liceaga", 06720, Mexico City, Mexico.
| | - Nathaly Rivera-Sotelo
- Cardiorespiratory Emergencies, Hospital General de México "Dr Eduardo Liceaga", 06720, Mexico City, Mexico.
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Kang J, Seo WJ, Lee EY, Chang SH, Choe J, Hong S, Song JW. Pleuroparenchymal fibroelastosis in rheumatoid arthritis-associated interstitial lung disease. Respir Res 2022; 23:143. [PMID: 35655303 PMCID: PMC9161503 DOI: 10.1186/s12931-022-02064-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2022] [Accepted: 05/23/2022] [Indexed: 12/17/2022] Open
Abstract
Background Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) featuring dense fibrosis of the visceral pleura and subpleural parenchyma, mostly in the upper lobes. PPFE can present in other ILDs, including rheumatoid arthritis-associated ILD (RA-ILD). The aim of this retrospective study was to investigate the prevalence and clinical implications of coexistent PPFE in RA-ILD. Methods Overall, 477 patients with RA-ILD were recruited from two cohorts; their clinical data and HRCT images were analysed. The criteria for diagnosing PPFE were (1) pleural thickening with bilateral subpleural dense fibrosis in the upper lobes, (2) evidence of disease progression, and (3) absence of other identifiable aetiologies. Results The median follow-up duration was 3.3 years. The mean age of the patients was 63.4 years, and 60.0% were women. PPFE was identified in 31 patients (6.5%). The PPFE group showed significantly lower body mass index and forced vital capacity (FVC) and more frequent usual interstitial pneumonia (UIP)-like pattern on HRCT than no-PPFE group. The risk factors for all-cause mortality were older age, lower FVC, and the presence of UIP-like pattern on HRCT; PPFE was not significantly associated with mortality in both all patients and a subgroup with a UIP-like pattern. The presence of PPFE was associated with a significantly increased risk of pneumothorax and greater decline in diffusing capacity. Conclusions PPFE was not rare in patients with RA-ILD and was significantly associated with an increased risk of pneumothorax and greater lung function decline, though we found no significant association with mortality. Supplementary Information The online version contains supplementary material available at 10.1186/s12931-022-02064-z.
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Affiliation(s)
- Jieun Kang
- Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Republic of Korea
| | - Woo Jung Seo
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea
| | - Eun Young Lee
- Division of Rheumatology, Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea
| | - Sung Hae Chang
- Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University Cheonan Hospital, Cheonan, Republic of Korea
| | - Jooae Choe
- Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Seokchan Hong
- Department of Rheumatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - Jin Woo Song
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul, 05505, South Korea.
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Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, Kreuter M, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nicholson AG, Ryerson CJ, Strek ME, Troy LK, Wijsenbeek M, Mammen MJ, Hossain T, Bissell BD, Herman DD, Hon SM, Kheir F, Khor YH, Macrea M, Antoniou KM, Bouros D, Buendia-Roldan I, Caro F, Crestani B, Ho L, Morisset J, Olson AL, Podolanczuk A, Poletti V, Selman M, Ewing T, Jones S, Knight SL, Ghazipura M, Wilson KC. Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med 2022; 205:e18-e47. [PMID: 35486072 PMCID: PMC9851481 DOI: 10.1164/rccm.202202-0399st] [Citation(s) in RCA: 1336] [Impact Index Per Article: 445.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
Background: This American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociación Latinoamericana de Tórax guideline updates prior idiopathic pulmonary fibrosis (IPF) guidelines and addresses the progression of pulmonary fibrosis in patients with interstitial lung diseases (ILDs) other than IPF. Methods: A committee was composed of multidisciplinary experts in ILD, methodologists, and patient representatives. 1) Update of IPF: Radiological and histopathological criteria for IPF were updated by consensus. Questions about transbronchial lung cryobiopsy, genomic classifier testing, antacid medication, and antireflux surgery were informed by systematic reviews and answered with evidence-based recommendations using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) approach. 2) Progressive pulmonary fibrosis (PPF): PPF was defined, and then radiological and physiological criteria for PPF were determined by consensus. Questions about pirfenidone and nintedanib were informed by systematic reviews and answered with evidence-based recommendations using the GRADE approach. Results:1) Update of IPF: A conditional recommendation was made to regard transbronchial lung cryobiopsy as an acceptable alternative to surgical lung biopsy in centers with appropriate expertise. No recommendation was made for or against genomic classifier testing. Conditional recommendations were made against antacid medication and antireflux surgery for the treatment of IPF. 2) PPF: PPF was defined as at least two of three criteria (worsening symptoms, radiological progression, and physiological progression) occurring within the past year with no alternative explanation in a patient with an ILD other than IPF. A conditional recommendation was made for nintedanib, and additional research into pirfenidone was recommended. Conclusions: The conditional recommendations in this guideline are intended to provide the basis for rational, informed decisions by clinicians.
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Fujisawa T, Horiike Y, Egashira R, Sumikawa H, Iwasawa T, Matsushita S, Sugiura H, Kataoka K, Hashisako M, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Enomoto N, Nakamura Y, Inui N, Suda T. Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias. Respir Res 2021; 22:290. [PMID: 34758816 PMCID: PMC8582158 DOI: 10.1186/s12931-021-01892-9] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2021] [Accepted: 11/07/2021] [Indexed: 11/10/2022] Open
Abstract
Background Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. Methods A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. Results Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. Conclusion Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice. Supplementary Information The online version contains supplementary material available at 10.1186/s12931-021-01892-9.
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Affiliation(s)
- Tomoyuki Fujisawa
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan.
| | - Yasuoki Horiike
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Ryoko Egashira
- Department of Radiology, Faculty of Medicine, Saga University, Saga, Japan
| | - Hiromitsu Sumikawa
- Department of Diagnostic Radiology, Sakai City Medical Center, Sakai, Japan
| | - Tae Iwasawa
- Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Shoichiro Matsushita
- Department of Radiology, St.Marianna University School of Medicine, Kawasaki, Japan
| | - Hiroaki Sugiura
- Department of Radiology, National Defense Medical College, Saitama, Japan
| | - Kensuke Kataoka
- Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Japan
| | - Mikiko Hashisako
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Hideki Yasui
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Hironao Hozumi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Masato Karayama
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Yuzo Suzuki
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Kazuki Furuhashi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Noriyuki Enomoto
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Yutaro Nakamura
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
| | - Naoki Inui
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan.,Department of Clinical Pharmacology and Therapeutics, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Takafumi Suda
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, 1-20-1 Handayama Higashi-ku, Hamamatsu, 431-3192, Japan
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Ikegami N, Nakajima N, Yoshizawa A, Handa T, Chen-Yoshikawa T, Kubo T, Tanizawa K, Ohsumi A, Yamada Y, Hamaji M, Nakajima D, Yutaka Y, Tanaka S, Watanabe K, Nakatsuka Y, Murase Y, Nakanishi T, Niwamoto T, Chin K, Date H, Hirai T. Clinical, radiological, and pathological features of idiopathic and secondary interstitial pneumonia cases with pleuroparenchymal fibroelastosis undergoing lung transplantation. Histopathology 2021; 80:665-676. [PMID: 34747513 DOI: 10.1111/his.14595] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2021] [Revised: 10/27/2021] [Accepted: 11/06/2021] [Indexed: 11/28/2022]
Abstract
AIMS Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare type of idiopathic interstitial pneumonia, and pathological PPFE is also observed in secondary interstitial pneumonia. This study aimed to evaluate the pathological findings associated with radiological PPFE-like lesions and the clinical and morphological features of patients with pathological PPFE. METHODS AND RESULTS We retrospectively reviewed the pathology of the explanted lungs from 59 lung transplant recipients with radiological PPFE-like lesions. Pathological PPFE lesions were identified in 14 patients with idiopathic cases and 12 patients with secondary cases. Pathological PPFE was associated with previous pneumothorax, a volume loss in the upper lobes and a flattened chest. Patients with idiopathic and secondary cases with pathological PPFE had similar clinical, radiological, and pathological findings, while fibroblastic foci were more common in patients with idiopathic cases, and patients with secondary cases more frequently showed alveolar septal thickening with elastosis or fibrosis. Post-transplantation survival did not differ between patients with idiopathic and secondary cases with pathological PPFE (log-rank; P=0.57) and was similar between patients with idiopathic cases with pathological PPFE and patients with idiopathic pulmonary fibrosis (IPF) (log-rank; P = 0.62). CONCLUSIONS Not all patients with interstitial pneumonia with radiological PPFE-like lesions have pathological PPFE. Characteristic clinical features can suggest the presence of pathological PPFE, and idiopathic and secondary cases with pathological PPFE are similar except for fibroblastic foci in idiopathic cases and alveolar septal thickening with elastosis or fibrosis in secondary cases. Patients with pathological PPFE have a similar prognosis to those with IPF after transplantation.
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Affiliation(s)
- Naoya Ikegami
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Naoki Nakajima
- Department of Diagnostic Pathology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akihiko Yoshizawa
- Department of Diagnostic Pathology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Tomohiro Handa
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of Advanced Medicine for Respiratory Failure, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Toyofumi Chen-Yoshikawa
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of Thoracic Surgery, Graduate School of Medicine, Nagoya University, Nagoya, Japan
| | - Takeshi Kubo
- Department of Diagnostic Imaging and Nuclear Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of Radiology, Tenri Hospital, Tenri, Japan
| | - Kiminobu Tanizawa
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akihiro Ohsumi
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Yoshito Yamada
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Masatsugu Hamaji
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Daisuke Nakajima
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Yojiro Yutaka
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Satona Tanaka
- Department of Thoracic Surgery, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Kizuku Watanabe
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,Department of Respiratory Medicine, Japanese Red Cross Wakayama Medical Center, Wakayama, Japan
| | - Yoshinari Nakatsuka
- Department of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Yuko Murase
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Tomoko Nakanishi
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan.,McGill International Collaborative School in Genomic Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Takafumi Niwamoto
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Kazuo Chin
- Department of Respiratory Care and Sleep Control Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hiroshi Date
- Department of Advanced Medicine for Respiratory Failure, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Toyohiro Hirai
- Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto, Japan
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Ikeda T, Kinoshita Y, Miyamura T, Ueda Y, Yoshida Y, Kushima H, Ishii H. Platythorax progresses with lung involvement in pleuroparenchymal fibroelastosis. Respir Investig 2021; 60:293-299. [PMID: 34732291 DOI: 10.1016/j.resinv.2021.09.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2021] [Revised: 09/28/2021] [Accepted: 09/29/2021] [Indexed: 01/17/2023]
Abstract
BACKGROUND Patients diagnosed with pleuroparenchymal fibroelastosis (PPFE) exhibit unique clinical features, including upper lobe-dominant lung involvement and platythorax (or flattened thoracic cage). Although platythorax have been shown to be a sign of disease progression, the temporal relationship between the progression of platythorax and the extent of lung involvement has not been closely investigated. METHODS We retrospectively investigated patients diagnosed with PPFE, who did not exhibit fibrotic lesions other than PPFE in the lower lobes. We estimated the fibrosis score, which is a visual score indicating the percentage of lung parenchyma occupied by the disease on computed tomography images selected every 2 cm from the lung apex to the lung base, and the flat chest index (the ratio of the anteroposterior diameter of the thoracic cage to the transverse diameter of the thoracic cage). Additionally, we investigated serial changes in the flat chest index and fibrosis score. RESULTS A total of 29 patients were included in this study. The fibrosis score was found to be weakly and inversely correlated with forced vital capacity %predicted at the diagnosis (r = -0.40, p = 0.038). Furthermore, the annual changes in the flat chest index and fibrosis score was found to be moderately and inversely correlated (r = -0.663, p = 0.0037). CONCLUSIONS These results indicate that there is a causal relationship between the progression of fibroelastosis and that of platythorax in patients with PPFE.
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Affiliation(s)
- Takato Ikeda
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Yoshiaki Kinoshita
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Takuto Miyamura
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Yusuke Ueda
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Yuji Yoshida
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Hisako Kushima
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan
| | - Hiroshi Ishii
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan.
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Nasser M, Si-Mohamed S, Turquier S, Traclet J, Ahmad K, Philit F, Bonniaud P, Chalabreysse L, Thivolet-Béjui F, Cottin V. Nintedanib in idiopathic and secondary pleuroparenchymal fibroelastosis. Orphanet J Rare Dis 2021; 16:419. [PMID: 34627338 PMCID: PMC8501734 DOI: 10.1186/s13023-021-02043-5] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2021] [Accepted: 09/19/2021] [Indexed: 11/10/2022] Open
Abstract
Background Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in patients with idiopathic and secondary PPFE. Patients admitted to a tertiary care center (2010–2019) were included into this retrospective analysis if they had a multidisciplinary diagnosis of PPFE, had been followed-up for 3 months or more, and had lung function tests and chest CTs available for review. Changes in pulmonary function tests were assessed using non-parametric tests and linear mixed effect model. Lung volumes were measured with lobar segmentation using chest CT. Results Out of 21 patients with PPFE, nine had received nintedanib, six had received another treatment and another six patients were monitored without drug therapy. Annual FVC (% of predicted) relative decline was − 13.6 ± 13.4%/year before nintedanib and − 1.6 ± 6.02%/year during nintedanib treatment (p = 0.014), whereas no significant change in FVC% relative decline was found in patients receiving another treatment (− 13.25 ± 34 before vs − 16.61 ± 36.2%/year during treatment; p = 0.343). Using linear mixed effect model, the slope in FVC was − 0.97%/month (95% CI: − 1.42; − 0.52) before treatment and − 0.50%/month (95% CI: − 0.88; 0.13) on nintedanib, with a difference between groups of + 0.47%/month (95% CI: 0.16; 0.78), p = 0.004. The decline in the upper lung volumes measured by CT was − 233 mL/year ± 387 mL/year before nintedanib and − 149 mL/year ± 173 mL/year on nintedanib (p = 0.327). Nintedanib tolerability was unremarkable. Conclusion In patients with PPFE, nintedanib treatment might be associated with slower decline in lung function, paving the way for prospective, controlled studies. Supplementary Information The online version contains supplementary material available at 10.1186/s13023-021-02043-5.
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Affiliation(s)
- Mouhamad Nasser
- Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, 28 avenue Doyen Lepine, 69677, Lyon, France
| | - Salim Si-Mohamed
- Radiology Department, Hospices Civils de Lyon, Lyon, France.,CREATIS, CNRS UMR 5220, INSERM U1206, INSA-Lyon, University Claude Bernard Lyon1, Lyon, France
| | - Ségolène Turquier
- Department of Respiratory Physiology, Hospices Civils de Lyon, Louis Pradel Hospital, Lyon, France
| | - Julie Traclet
- Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, 28 avenue Doyen Lepine, 69677, Lyon, France
| | - Kaïs Ahmad
- Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, 28 avenue Doyen Lepine, 69677, Lyon, France
| | - François Philit
- Department of Respiratory Medicine, Croix Rousse Hospital, Hospices Civil de Lyon, Lyon, France
| | - Philippe Bonniaud
- Department of Pulmonary Medicine and Intensive Care Unit, Constitutive Reference Center for Rare Pulmonary Diseases, François Mitterrand Teaching Hospital, Inserm U1231, University Bourgogne-Franche Comté, Dijon, France
| | - Lara Chalabreysse
- Department of Pathology, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.,UMR754, INRAE, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France
| | - Françoise Thivolet-Béjui
- Department of Pathology, Louis Pradel Hospital, Hospices Civils de Lyon, Lyon, France.,UMR754, INRAE, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France
| | - Vincent Cottin
- Department of Respiratory Medicine, National Coordinating Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, 28 avenue Doyen Lepine, 69677, Lyon, France. .,UMR754, INRAE, Université Claude Bernard Lyon 1, Université de Lyon, Lyon, France.
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Gudmundsson E, Zhao A, Mogulkoc N, Stewart I, Jones MG, Van Moorsel CH, Savas R, Brereton CJ, Van Es HW, Unat O, Pontoppidan K, Van Beek F, Veltkamp M, Gholipour B, Nair A, Wells AU, Janes SM, Alexander DC, Jacob J. Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment. EClinicalMedicine 2021; 38:101009. [PMID: 34505028 PMCID: PMC8413236 DOI: 10.1016/j.eclinm.2021.101009] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2021] [Revised: 06/10/2021] [Accepted: 06/16/2021] [Indexed: 11/17/2022] Open
Abstract
BACKGROUND Idiopathic pulmonary fibrosis (IPF) and pleuroparenchymal fibroelastosis (PPFE) are known to have poor outcomes but detailed examinations of prognostic significance of an association between these morphologic processes are lacking. METHODS Retrospective observational study of independent derivation and validation cohorts of IPF populations. Upper-lobe PPFE extent was scored visually (vPPFE) as categories of absent, moderate, marked. Computerised upper-zone PPFE extent (cPPFE) was examined continuously and using a threshold of 2·5% pleural surface area. vPPFE and cPPFE were evaluated against 1-year FVC decline (estimated using mixed-effects models) and mortality. Multivariable models were adjusted for age, gender, smoking history, antifibrotic treatment and diffusion capacity for carbon monoxide. FINDINGS PPFE prevalence was 49% (derivation cohort, n = 142) and 72% (validation cohort, n = 145). vPPFE marginally contributed 3-14% to variance in interstitial lung disease (ILD) severity across both cohorts.In multivariable models, marked vPPFE was independently associated with 1-year FVC decline (derivation: regression coefficient 18·3, 95 CI 8·47-28·2%; validation: 7·51, 1·85-13·2%) and mortality (derivation: hazard ratio [HR] 7·70, 95% CI 3·50-16·9; validation: HR 3·01, 1·33-6·81). Similarly, continuous and dichotomised cPPFE were associated with 1-year FVC decline and mortality (cPPFE ≥ 2·5% derivation: HR 5·26, 3·00-9·22; validation: HR 2·06, 1·28-3·31). Individuals with cPPFE ≥ 2·5% or marked vPPFE had the lowest median survival, the cPPFE threshold demonstrated greater discrimination of poor outcomes at two and three years than marked vPPFE. INTERPRETATION PPFE quantification supports distinction of IPF patients with a worse outcome independent of established ILD severity measures. This has the potential to improve prognostic management and elucidate separate pathways of disease progression. FUNDING This research was funded in whole or in part by the Wellcome Trust [209,553/Z/17/Z] and the NIHR UCLH Biomedical Research Centre, UK.
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Affiliation(s)
- Eyjolfur Gudmundsson
- Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom
| | - An Zhao
- Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom
| | - Nesrin Mogulkoc
- Department of Respiratory Medicine, Ege University Hospital, Izmir, Turkey
| | - Iain Stewart
- National Heart & Lung Institute, Imperial College London, London, United Kingdom
| | - Mark G. Jones
- NIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom
| | - Coline H.M. Van Moorsel
- Department of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the Netherlands
| | - Recep Savas
- Department of Radiology, Ege University Hospital, Izmir, Turkey
| | - Christopher J. Brereton
- NIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom
| | - Hendrik W. Van Es
- Department of Radiology, St Antonius Hospital, Nieuwegein, the Netherlands
| | - Omer Unat
- Department of Respiratory Medicine, Ege University Hospital, Izmir, Turkey
| | - Katarina Pontoppidan
- NIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United Kingdom
| | - Frouke Van Beek
- Department of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the Netherlands
| | - Marcel Veltkamp
- Department of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the Netherlands
- Division of Heart and Lungs, University Medical Center, Utrecht, the Netherlands
| | - Bahareh Gholipour
- Department of Radiology, University College London Hospitals NHS Foundation Trust, London, United Kingdom
| | - Arjun Nair
- Department of Radiology, University College London Hospitals NHS Foundation Trust, London, United Kingdom
| | - Athol U. Wells
- Interstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, United Kingdom
| | - Sam M. Janes
- Lungs for Living Research Centre, UCL, London, United Kingdom
| | - Daniel C. Alexander
- Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom
| | - Joseph Jacob
- Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom
- Lungs for Living Research Centre, UCL, London, United Kingdom
- Corresponding author at: Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom.
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d'Alessandro M, Bellisai F, Bergantini L, Cameli P, D'Alessandro R, Mazzei MA, Gentili F, Conticini E, Selvi E, Frediani B, Matucci-Cerinic M, Bargagli E. Prognostic role of KL-6 in SSc-ILD patients with pleuroparenchymal fibroelastosis. Eur J Clin Invest 2021; 51:e13543. [PMID: 33759179 DOI: 10.1111/eci.13543] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/12/2021] [Revised: 03/08/2021] [Accepted: 03/12/2021] [Indexed: 02/06/2023]
Abstract
BACKGROUND Krebs von den Lungen-6 (KL-6) is a high-molecular-weight (200kDa) glycoprotein proposed as a diagnostic biomarker for differentiating interstitial lung disease (ILD). Systemic sclerosis (SSc) is a rare immune-mediated disorder, and ILD is the leading cause of morbidity and mortality. Pleuroparenchymal fibroelastosis (PPFE) has been described to have a poor prognosis in SSc-ILD patients. This study undertook to compare serial changes in KL-6 in SSc-ILD patients with and without PPFE, to verify its prognostic value as a disease biomarker. MATERIALS AND METHODS Twenty-five SSc-ILD patients (median IQR, 62 (56-58); 20% males) were retrospectively enrolled. 12 SSc-ILD patients (48%) had also a radiological diagnosis of PPFE. Serum KL-6 concentrations were measured by KL-6 reagent assay (Fujirebio Europe, Ghent, Belgium). RESULTS Serum KL-6 measurements were increased in SSc-ILD patients with and without PPFE compared with healthy controls (P < .0001). Comparative analysis of the rate of variation of KL-6 over the 6 years of follow-up was performed by serial two-yearly KL-6 measurements: Δ1(t1-t0), Δ2(t2-t1) and Δ3(t3-t2). In SSc-ILD patients with PPFE pattern, Δ3 was significantly different than those without PPFE pattern (P = .0020). Serum KL-6 levels were significantly different (P = .0455) either at Δ2 and Δ3 in the PPFE group. In SSc-ILD patients with PPFE, at t3 serum KL-6 concentrations were inversely correlated with FEV1 (r = -.76; P = .037) and FVC percentages (r = -.79; P = .028). CONCLUSION These results suggest that serial measurements of KL-6 in the follow-up of these patients may help to monitor disease progression. In real life, in SSc-ILD patients PPFE should be always evaluated at CT and when present should suggest a tight follow-up to monitor its evolution.
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Affiliation(s)
- Miriana d'Alessandro
- Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy
| | - Francesca Bellisai
- Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy
| | - Laura Bergantini
- Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy
| | - Paolo Cameli
- Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy
| | - Roberto D'Alessandro
- Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy
| | - Maria Antonietta Mazzei
- Unit of Diagnostic Imaging, Department of Medical, Surgical and Neurosciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Tuscany, Italy
| | - Francesco Gentili
- Unit of Diagnostic Imaging, Department of Medical, Surgical and Neurosciences and of Radiological Sciences, University of Siena, Azienda Ospedaliero-Universitaria Senese, Siena, Tuscany, Italy
| | - Edoardo Conticini
- Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy
| | - Enrico Selvi
- Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy
| | - Bruno Frediani
- Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy
| | - Marco Matucci-Cerinic
- Department of Experimental and Clinical Medicine, Division of Rheumatology, University of Florence, Florence, Italy
| | - Elena Bargagli
- Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy
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Kinoshita Y, Ikeda T, Miyamura T, Ueda Y, Yoshida Y, Kushima H, Fujita M, Ogura T, Watanabe K, Ishii H. A proposed prognostic prediction score for pleuroparenchymal fibroelastosis. Respir Res 2021; 22:215. [PMID: 34330287 PMCID: PMC8400711 DOI: 10.1186/s12931-021-01810-z] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2021] [Accepted: 07/17/2021] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Clinical course of pleuroparenchymal fibroelastosis (PPFE) shows considerable variation among patients, but there is no established prognostic prediction model for PPFE. METHODS The prediction model was developed using retrospective data from two cohorts: our single-center cohort and a nationwide multicenter cohort involving 21 institutions. Cox regression analyses were used to identify prognostic factors. The total score was defined as the weighted sum of values for the selected variables. The performance of the prediction models was evaluated by Harrell's concordance index (C-index). We also examined the usefulness of the gender-age-physiology (GAP) model for predicting the prognosis of PPFE patients. RESULTS We examined 104 patients with PPFE (52 cases from each cohort). In a multivariate Cox analysis, a lower forced vital capacity (FVC [defined as FVC < 65%]; hazard ratio [HR], 2.23), a history of pneumothorax (HR, 3.27), the presence of a lower lobe interstitial lung disease (ILD) (HR, 2.31), and higher serum Krebs von den Lungen-6 (KL-6) levels (> 550 U/mL, HR, 2.56) were significantly associated with a poor prognosis. The total score was calculated as 1 × (FVC, < 65%) + 1 × (history of pneumothorax) + 1 × (presence of lower lobe ILD) + 1 × (KL-6, > 550 U/mL). PPFE patients were divided into three groups based on the prognostic score: stage I (0-1 points), stage II (2 points), and stage III (3-4 points). The survival rates were significantly different in each stage. The GAP stage was significantly associated with the prognosis of PPFE, but no difference was found between moderate (stage II) and severe (stage III) disease. Our new model for PPFE patients (PPFE Prognosis Score) showed better performance in the prediction of mortality in comparison to the GAP model (C-index of 0.713 vs. 0.649). CONCLUSIONS Our new model for PPFE patients could be useful for predicting their prognosis.
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Affiliation(s)
- Yoshiaki Kinoshita
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Takato Ikeda
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Takuto Miyamura
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Yusuke Ueda
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Yuji Yoshida
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Hisako Kushima
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan
| | - Masaki Fujita
- Department of Respiratory Medicine, Fukuoka University Hospital, Fukuoka, Japan
| | - Takashi Ogura
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Kanagawa, Japan
| | - Kentaro Watanabe
- Department of Respiratory Medicine, Nishi Fukuoka Hospital, Fukuoka, Japan
| | - Hiroshi Ishii
- Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, 1-1-1 Zokumyoin, Chikushino, Fukuoka, 818-8502, Japan.
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Konopka KE, Myers JL. Interstitial lung disease pathology in systemic sclerosis. Ther Adv Musculoskelet Dis 2021; 13:1759720X211032437. [PMID: 34349846 PMCID: PMC8287363 DOI: 10.1177/1759720x211032437] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2021] [Accepted: 06/25/2021] [Indexed: 12/11/2022] Open
Abstract
Interstitial lung disease is a relatively frequent manifestation of systemic
sclerosis with approximately one-third of patients developing clinical
restrictive lung disease. Fibrotic nonspecific interstitial pneumonia is the
most common cause of diffuse parenchymal lung disease in patients with systemic
sclerosis-associated interstitial lung disease (SSc-ILD), followed by usual
interstitial pneumonia (UIP). Radiographic pleuroparenchymal fibroelastosis-like
changes may accompany other forms of interstitial lung disease, most commonly
UIP. In an appropriate clinical setting with supportive high-resolution computed
tomography findings, lung biopsy is not needed to confirm the presence of
interstitial lung disease and surgical lung biopsies are often reserved for
atypical presentations. In this review, we discuss the histological findings
that define the most common patterns of SSc-ILD and outline other findings
sometimes encountered in lung biopsies obtained from systemic sclerosis
patients, including pulmonary vascular changes, aspiration, chronic pleuritis,
and diffuse alveolar damage.
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Affiliation(s)
- Kristine E Konopka
- Department of Pathology, University of Michigan, Michigan Medicine, 2800 Plymouth Road, Building 35, Ann Arbor, MI 48109, USA
| | - Jeffrey L Myers
- Department of Pathology, University of Michigan, Ann Arbor, MI, USA
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Sekine A, Hagiwara E, Iwasawa T, Otoshi R, Erina T, Shintani R, Okabayashi H, Ikeda S, Oda T, Okuda R, Kitamura H, Baba T, Komatsu S, Ogura T. Asbestos exposure and tuberculous pleurisy as developmental causes of progressive unilateral upper-lung field pulmonary fibrosis radiologically consistent with pleuroparenchymal fibroelastosis. Respir Investig 2021; 59:837-844. [PMID: 34172419 DOI: 10.1016/j.resinv.2021.05.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2021] [Revised: 05/18/2021] [Accepted: 05/20/2021] [Indexed: 11/28/2022]
Abstract
BACKGROUND Unilateral upper-lung field pulmonary fibrosis (upper-PF), which is radiologically consistent with pleuroparenchymal fibroelastosis, develops after thoracic surgery. In most patients with unilateral upper-PF, aberrant intra-/extra thoracic air commonly emerges and an autopsy shows chronic pleuritis, which indicates that pleural involvement is associated with upper-PF development. If so, there may be patients with unilateral upper-PF who have a history of pleural involvement, including tuberculous pleurisy (TP) or asbestos exposure (AE). This study aimed to examine this supposition. METHODS We examined the radiological reports of all consecutive patients from 2012 to 2018 to investigate whether there were patients having unilateral upper-PF and a history of TP or AE. RESULTS Eight patients were included in the study. Five patients had a history of TP, and the remaining three had that of AE. All patients were men and had respiratory symptoms, and seven patients presented with restrictive ventilatory impairment. The interval between TP or last AE and upper-PF development was long, with a median of over 20 years. The upper-PF lesion was commonly located in the right lung, and aberrant intrathoracic air was observed in five patients during their clinical course. Additionally, the upper-PF lesion transformed into a cystic lesion in six patients, which resulted in Aspergillus infection in two patients. The prognosis was poor, with a median overall survival of 38 months. CONCLUSIONS Unilateral upper-PF developed even in patients with a history of pleural involvement. Our results indicate that pleural involvement plays an important role in the development of unilateral upper-PF.
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Affiliation(s)
- Akimasa Sekine
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan.
| | - Eri Hagiwara
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Tae Iwasawa
- Department of Radiology, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Ryota Otoshi
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Tabata Erina
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Ryota Shintani
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Hiroko Okabayashi
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Satoshi Ikeda
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Tsuneyuki Oda
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Ryo Okuda
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Hideya Kitamura
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Tomohisa Baba
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Shigeru Komatsu
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
| | - Takashi Ogura
- Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Yokohama, Japan
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Ryerson CJ, Corte TJ, Myers JL, Walsh SLF, Guler SA. A contemporary practical approach to the multidisciplinary management of unclassifiable interstitial lung disease. Eur Respir J 2021; 58:13993003.00276-2021. [PMID: 34140296 PMCID: PMC8674517 DOI: 10.1183/13993003.00276-2021] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Accepted: 05/04/2021] [Indexed: 11/05/2022]
Abstract
Fibrotic interstitial lung diseases (ILDs) frequently have nonspecific and overlapping clinical and radiological features, resulting in approximately 10-20% of patients with ILD lacking a clear diagnosis and thus being labelled with unclassifiable ILD. The objective of this review is to describe how patients with unclassifiable ILD should be evaluated and what impact specific clinical, radiological, and histopathological features may have on management decisions, focusing on patients with a predominantly fibrotic phenotype. We highlight recent data that have suggested an increasing role for antifibrotic medications in a variety of fibrotic ILDs, but justify the ongoing importance of making an accurate ILD diagnosis given the benefit of immunomodulatory therapies in many patient populations. We provide a practical approach to support management decisions that can be used by clinicians and tested by clinical researchers, and further identify the need for additional research to support a rational and standardised approach to the management of patients with unclassifiable ILD.
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Affiliation(s)
- Christopher J Ryerson
- Department of Medicine, University of British Columbia and Centre for Heart Lung Innovation, St. Paul"s Hospital, Vancouver, Canada
| | - Tamera J Corte
- Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney; University of Sydney; Centre of Research Excellence for Pulmonary Fibrosis, Sydney, Australia
| | - Jeffrey L Myers
- Department of Pathology, Michigan Medicine, Ann Arbor, Michigan, United States
| | - Simon L F Walsh
- National Heart and Lung Institute, Imperial College, London, United Kingdom
| | - Sabina A Guler
- Department of Pulmonary Medicine, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
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Pulmonary Fibroelastotic Remodelling Revisited. Cells 2021; 10:cells10061362. [PMID: 34205982 PMCID: PMC8227669 DOI: 10.3390/cells10061362] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/21/2021] [Revised: 05/26/2021] [Accepted: 05/28/2021] [Indexed: 12/27/2022] Open
Abstract
Pulmonary fibroelastotic remodelling occurs within a broad spectrum of diseases with vastly divergent outcomes. So far, no comprehensive terminology has been established to adequately address and distinguish histomorphological and clinical entities. We aimed to describe the range of fibroelastotic changes and define stringent histological criteria. Furthermore, we wanted to clarify the corresponding terminology in order to distinguish clinically relevant variants of pulmonary fibroelastotic remodelling. We revisited pulmonary specimens with fibroelastotic remodelling sampled during the last ten years at a large European lung transplant centre. Consensus-based definitions of specific variants of fibroelastotic changes were developed on the basis of well-defined cases and applied. Systematic evaluation was performed in a steps-wise algorithm, first identifying the fulcrum of the respective lesions, and then assessing the morphological changes, their distribution and the features of the adjacent parenchyma. We defined typical alveolar fibro-elastosis as collagenous effacement of the alveolar spaces with accompanying hyper-elastosis of the remodelled and paucicellular alveolar walls, independent of the underlying disease in 45 cases. Clinically, this pattern could be seen in (idiopathic) pleuroparenchymal fibro-elastosis, interstitial lung disease with concomitant alveolar fibro-elastosis, following hematopoietic stem cell and lung transplantation, autoimmune disease, radio-/chemotherapy, and pulmonary apical caps. Novel in-transit and activity stages of fibroelastotic remodelling were identified. For the first time, we present a comprehensive definition of fibroelastotic remodelling, its anatomic distribution, and clinical associations, thereby providing a basis for stringent patient stratification and prediction of outcome.
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Morshid A, Moshksar A, Das A, Duarte AG, Palacio D, Villanueva-Meyer J. HRCT Diagnosis of Pleuroparenchymal fibroelastosis: Report of two cases. Radiol Case Rep 2021; 16:1564-1569. [PMID: 33981378 PMCID: PMC8085788 DOI: 10.1016/j.radcr.2021.03.051] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2021] [Revised: 03/23/2021] [Accepted: 03/25/2021] [Indexed: 11/17/2022] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonia that is often underdiagnosed on computed tomography scans. The disease process involves a combination of fibrosis involving the visceral pleura and fibroelastic changes within the subpleural lung parenchyma. Although definitive diagnosis is based on pathological evaluation, this is often not feasible and pattern recognition on CT as "definite PPFE" or "consistent with PPFE" is important given that sub group of patients will undergo rapid progression with clinical deterioration.
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Affiliation(s)
- Ali Morshid
- Department of Diagnostic Radiology, University of Texas Medical Branch, Galveston TX 77555
| | - Amin Moshksar
- Department of Diagnostic Radiology, University of Texas Medical Branch, Galveston TX 77555
| | - Aparna Das
- Department of Internal Medicine, University of Texas Medical Branch, Galveston TX 77555
| | - Alexander G Duarte
- Department of Internal Medicine, University of Texas Medical Branch, Galveston TX 77555
| | - Diana Palacio
- Department of Diagnostic Radiology, University of Texas Medical Branch, Galveston TX 77555
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Ricoy J, Suárez-Antelo J, Antúnez J, Martínez de Alegría A, Ferreiro L, Toubes ME, Casal A, Valdés L. Pleuroparenchymal fibroelastosis: Clinical, radiological and histopathological features. Respir Med 2021; 191:106437. [PMID: 33992495 DOI: 10.1016/j.rmed.2021.106437] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/20/2021] [Revised: 04/10/2021] [Accepted: 04/21/2021] [Indexed: 01/24/2023]
Abstract
Pleuroparenchymal fibroelastosis (PPFE) is a rare, generally idiopathic form of interstitial pneumonia with unique clinical, radiological and histopathological features. It is named after the presence of upper lobe pleural and subjacent parenchymal fibrosis, with accompanying elastic fibers. Although it is usually an idiopathic disease, it has been linked to other co-existent diseases. Diagnostic suspicion of PPFE is based on the identification of typical abnormalities on chest CT scan, which are prevailingly located in the upper lobes, adjacent to the apex of the lungs. Diagnosis can be confirmed by histological analysis, although biopsy is not always feasible. The disease is generally progressive, but not uniformly. The course of the disease is frequently slow and involves a progressive loss of upper lobe volume, which results in platythorax, associated with a significant reduction of body mass. PPFE concomitant to other interstitial lung diseases is associated with a poorer prognosis. The disease occasionally progresses rapidly causing irreversible respiratory insufficiency, which leads to death. Currently, there is no effective pharmacological therapy available, and lung transplantation is the best therapeutic option. The purpose of this review is to draw the attention to PPFE, describe its clinical, radiological and histopathological features, analyze its diagnostic criteria, and provide an update on the management of the disease.
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Affiliation(s)
- Jorge Ricoy
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - Juan Suárez-Antelo
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - José Antúnez
- Department of Pathology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - Anxo Martínez de Alegría
- Department of of Radiology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - Lucía Ferreiro
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain; Multidisciplinary Research Group on Pulmonology, Health Research Institute of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.
| | - María Elena Toubes
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - Ana Casal
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain.
| | - Luis Valdés
- Department of Pulmonology, Complejo Hospitalario Clínico-Universitario de Santiago, Santiago de Compostela, Spain; Multidisciplinary Research Group on Pulmonology, Health Research Institute of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.
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Prognostic and Clinical Value of Cluster Analysis in Idiopathic Pleuroparenchymal Fibroelastosis Phenotypes. J Clin Med 2021; 10:jcm10071498. [PMID: 33916508 PMCID: PMC8038478 DOI: 10.3390/jcm10071498] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/27/2021] [Revised: 03/26/2021] [Accepted: 04/01/2021] [Indexed: 02/07/2023] Open
Abstract
Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a distinctive interstitial pneumonia with upper lobe predominance that shows unique morphological features among idiopathic interstitial pneumonias (IIPs). Affected patients have a variety of clinical presentations with heterogeneous clinical courses. Cluster analysis is a valuable tool for identifying distinct clinical phenotypes under heterogeneous conditions. This study aimed to identify the phenotypes of patients with idiopathic PPFE. Using cluster analysis, novel PPFE phenotypes were identified among subjects from our multicenter cohort, and outcomes were stratified according to phenotypic clusters. Among the subjects with baseline data (N = 84), four clusters were identified. Cluster 1 included younger male subjects with coexisting non-UIP-like patterns. Cluster 2 included elderly female nonsmokers with low body mass index (BMI). Cluster 3 included elderly male smokers with a coexisting IP-like pattern. Cluster 4 included younger male smokers without lower lobe lesions. Patients in cluster 3 had significantly worse survival outcomes than those in clusters 1, 2, and 4 (p < 0.001, p = 0.0041, and p = 0.0155, respectively). Among idiopathic PPFE patients, cluster analysis using baseline characteristics identified four distinct clinical phenotypes that might predict survival outcomes.
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Shiiya H, Sato M. Lung Transplantation for Pleuroparenchymal Fibroelastosis. J Clin Med 2021; 10:jcm10050957. [PMID: 33804467 PMCID: PMC7957581 DOI: 10.3390/jcm10050957] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2021] [Revised: 02/20/2021] [Accepted: 02/20/2021] [Indexed: 12/26/2022] Open
Abstract
Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients.
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Affiliation(s)
- Haruhiko Shiiya
- Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan;
- Department of Cardiovascular and Thoracic Surgery, Hokkaido University Graduate School of Medicine, Kita 15 Nishi 7, Kita-ku, Sapporo 060-8638, Hokkaido, Japan
| | - Masaaki Sato
- Department of Thoracic Surgery, The University of Tokyo Graduate School of Medicine, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan;
- Correspondence: ; Tel.: +81-3-3815-5411; Fax: +81-3-5800-9156
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45
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Lee JH, Chae EJ, Song JS, Kim M, Song JW. Pleuroparenchymal fibroelastosis in Korean patients: clinico-radiologic-pathologic features and 2-year follow-up. Korean J Intern Med 2021; 36:S132-S141. [PMID: 32088939 PMCID: PMC8009146 DOI: 10.3904/kjim.2019.303] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2019] [Revised: 10/25/2019] [Accepted: 12/17/2019] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND/AIMS Pleuroparenchymal fibroelastosis (PPFE) is a type of rare idiopathic interstitial pneumonia that is characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis. This study aimed to determine the clinico-radiologic-pathologic features and prognosis of Korean patients with PPFE. METHODS A total of 26 patients who were confirmed to have PPFE by lung biopsy, were included, and their clinico-radiologic-pathologic findings were retrospectively analyzed. RESULTS The mean follow-up period was 23.8 months, and the mean age of the patients was 62.5 years; 61.5% were men and 50% were smokers. Cough and dyspnea were the most frequent presenting symptoms, and restrictive pattern was the most common observation in lung function. In 84.6% of the subjects, lower lobe involvement was found on chest computed tomography, and the usual interstitial pneumonia (UIP) pattern was the most common (59.1%). Among patients whose lower lobe was biopsied (n = 13), the UIP pattern was the most common (46.2%). Patients with lower lobe involvement were older and walked a shorter distance during the 6-minute walk test, compared to those without. Spontaneous pneumothorax was a common complication (26.9%), and 15.4% of the patients died mostly due to pneumonia (100%). The 1- and 3-year survival rates were 90.2% and 84.5%, respectively. CONCLUSION Clinical features of Korean patients with PPFE were similar to those reported previously; however, lower lobe involvement was more frequent. During follow-up, one-fourth of the patients experienced pneumothorax and one-fifth died from pneumonia.
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Affiliation(s)
- Jae Ha Lee
- Division of Pulmonology and Critical Care Medicine, Department of Internal Medicine, Inje niversity Haeundae Paik Hospital, Busan, Korea
| | - Eun Jin Chae
- Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Joon Seon Song
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Miae Kim
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Jin Woo Song
- Department of Pulmonary and Critical Care Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Sugino K, Ono H, Shimizu H, Kurosawa T, Matsumoto K, Ando M, Mori K, Tsuboi E, Homma S, Kishi K. Treatment with antifibrotic agents in idiopathic pleuroparenchymal fibroelastosis with usual interstitial pneumonia. ERJ Open Res 2021; 7:00196-2020. [PMID: 33681342 PMCID: PMC7917230 DOI: 10.1183/23120541.00196-2020] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2020] [Accepted: 11/05/2020] [Indexed: 11/10/2022] Open
Abstract
Background There are no established therapeutic options available for idiopathic pleuroparenchymal fibroelastosis (IPPFE) apart from supportive care and lung transplantation. Furthermore, it is known that IPPFE with a usual interstitial pneumonia (UIP) pattern and lower lobe predominance is a disease entity distinct from idiopathic pulmonary fibrosis (IPF). To our knowledge, few studies are available that report on the efficacy of antifibrotic agents for IPPFE with UIP. Aim The aim of this study was to compare the efficacy of antifibrotic agents between IPPFE with UIP and typical IPF in real-world clinical practice. Patients and methods A retrospective analysis was performed on the medical records of all patients at two interstitial lung disease centres. Sixty-four patients were diagnosed as having IPPFE with UIP and 195 patients were diagnosed with typical IPF. We compared the efficacy of antifibrotic agents between these two groups. Results Survival time was significantly shorter in the patients with IPPFE with UIP. Some 125 patients were administered antifibrotic agents for over 6 months (34 with IPPFE with UIP and 91 with typical IPF). Reduced forced vital capacity (FVC) 6 months after treatment with antifibrotic agents was significantly greater in the IPPFE with UIP group than in those in the typical IPF group. Moreover, the change in % predicted FVC was significantly greater during the follow-up in patients with IPPFE with UIP compared with those with typical IPF. Conclusions The efficacy of antifibrotic agents was limited in patients with IPPFE with UIP. Thus, IPPFE with UIP remains a fatal and progressive disease. This study demonstrated that patients with IPPFE with UIP have a poorer prognosis than those with typical IPF because these patients were intractable to treatment with antifibrotic agentshttps://bit.ly/38LbAKD
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Affiliation(s)
- Keishi Sugino
- Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan.,Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Hirotaka Ono
- Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
| | - Hiroshige Shimizu
- Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Takeyuki Kurosawa
- Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
| | - Keiko Matsumoto
- Dept of Radiology, Toho University Omori Medical Center, Tokyo, Japan
| | - Masahiro Ando
- Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
| | - Kiyoshi Mori
- Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
| | - Eiyasu Tsuboi
- Dept of Respiratory Medicine, Tsuboi Hospital, Koriyama city, Fukushima, Japan
| | - Sakae Homma
- Dept of Advanced and Integrated Interstitial Lung Diseases Research, School of Medicine, Toho University, Tokyo, Japan
| | - Kazuma Kishi
- Dept of Respiratory Medicine, Toho University Omori Medical Center, Tokyo, Japan
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Alveolar Epithelial Denudation Is a Major Factor in the Pathogenesis of Pleuroparenchymal Fibroelastosis. J Clin Med 2021; 10:jcm10050895. [PMID: 33668178 PMCID: PMC7956653 DOI: 10.3390/jcm10050895] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2021] [Revised: 02/14/2021] [Accepted: 02/19/2021] [Indexed: 01/22/2023] Open
Abstract
The pathogenesis of pleuroparenchymal fibroelastosis (PPFE), a rare interstitial lung disease, remains unclear. Based on previous reports and our experience, we hypothesized that alveolar epithelial denudation (AED) was involved in the pathogenesis of PPFE. This multicenter retrospective study investigated the percentage of AED and the features of the denudated areas in 26 PPFE cases, 30 idiopathic pulmonary fibrosis (IPF) cases, and 29 controls. PPFE patients had lower forced vital capacities and higher residual volume/total lung capacities in pulmonary function tests compared to IPF and control patients. Histopathologically, subpleural fibroelastosis was observed in PPFE, and AED was observed in 12.01% of cases in the subpleural or interlobular septa regardless of fibroelastosis. The percentage of AED in the PPFE group was significantly higher than that in the IPF group (6.84%; p = 0.03) and the normal group (1.19%; p < 0.001). In the IPF group, the percentage of AED and the presence of PPFE-like lesions in the upper lobes were examined radiologically, but no correlation was found. We showed that AED frequently occurred in PPFE. AED was less frequent in IPF, which, in combination with imaging data, suggests that PPFE may have a different pathogenesis from IPF.
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Comparison of Clinical Characteristics and Outcomes between Idiopathic and Secondary Pleuroparenchymal Fibroelastosis. J Clin Med 2021; 10:jcm10040846. [PMID: 33670776 PMCID: PMC7922642 DOI: 10.3390/jcm10040846] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2021] [Revised: 02/12/2021] [Accepted: 02/15/2021] [Indexed: 12/14/2022] Open
Abstract
Background: Pleuroparenchymal fibroelastosis (PPFE) is a unique clinical, radiologic, and histopathologic entity for which several potential etiologies have been reported recently. However, there has been no comprehensive study of secondary PPFE. Objective: Assessment of the clinical characteristics, outcomes, and prognostic factors of secondary and idiopathic PPFE. Methods: We retrospectively reviewed the medical records of consecutive PPFE patients between January 1999 and December 2018. We identified 132 idiopathic PPFE patients and 32 secondary PPFE patients. Results: The incidence of interstitial lung disease (ILD) pattern different from the usual interstitial pneumonia (UIP) pattern in the lower lobes was higher in secondary PPFE patients (38.5%) than in idiopathic PPFE patients (61.5%, p = 0.02). The idiopathic and secondary PPFE groups did not differ significantly in terms of laboratory data, respiratory complications, and survival (median: 5.0 years vs. 4.1 years, p = 0.95). The presence of UIP pattern was independently associated with increased mortality in multivariate analyses in idiopathic PPFE patients, but not in secondary PPFE patients. Conclusions: The frequency and prognostic impact of UIP-pattern ILD differed between idiopathic and secondary PPFE patients. Lung transplantation should be considered in secondary PPFE patients with low diffusing capacity of the lungs for carbon monoxide (DLCO) regardless of lower-lobe ILD pattern.
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Orlandi M, Landini N, Bruni C, Sambataro G, Nardi C, Bargagli E, Tomassetti S, Occhipinti M, Bellando Randone S, Guiducci S, Vancheri C, Colagrande S, Matucci-Cerinic M. Pleuroparenchymal fibroelastosis in rheumatic autoimmune diseases: a systematic literature review. Rheumatology (Oxford) 2021; 59:3645-3656. [PMID: 33313932 DOI: 10.1093/rheumatology/keaa451] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2020] [Revised: 06/08/2020] [Accepted: 06/30/2020] [Indexed: 01/07/2023] Open
Abstract
OBJECTIVES Pleuroparenchymal fibroelastosis (PPFE) is characterized by predominantly upper lobe pleural and subjacent parenchymal fibrosis; PPFE features were described in patients with rheumatic autoimmune diseases (RAID). A systematic literature review was performed to investigate the prevalence, prognosis and potential association of PPFE with previous immunosuppression in RAID. METHODS EMBASE, Web of Science and PubMed databases were questioned from inception to 1 September 2019. Articles published in English and addressing PPFE in patients with RAID were selected. RESULTS Twenty out of 794 papers were selected with a total of 76 cases of RAID-PPFE patients (20 SSc, 9 RA, 6 IIM6 primary SS, 5 overlap syndromes, 3 ANCA-associated vasculitides, 2 granulomatosis with polyangiitis, 1 microscopic polyangiitis, 1 UCTD, 1 SLE, 1 GCA and 21 patients with non-specified RAID). Dyspnoea was the most frequently reported symptom (37/48 patients, 77%). Patients frequently presented with a restrictive pattern and decline in diffusing lung capacity for carbon monoxide. During the follow-up, 7/12 patients had progression at imaging, 22/39 presented a generic clinical worsening, 19/38 had a functional deterioration and 15/43 remained stable. CONCLUSION The present systematic literature review confirms that PPFE features are present in RAID. Rheumatologists should be aware of this new radiological pattern that holds a bad prognosis.
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Affiliation(s)
- Martina Orlandi
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence
| | - Nicholas Landini
- Department of Radiology, Ca' Foncello Regional Hospital, Treviso.,Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit no. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Florence
| | - Cosimo Bruni
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence
| | - Gianluca Sambataro
- Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Disease, A.O.U. 'Policlinico-Vittorio Emanuele', Catania
| | - Cosimo Nardi
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit no. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Florence
| | - Elena Bargagli
- Department of Medicine, Surgery and Neurosciences, Respiratory Diseases and Lung Transplantation, Regional Referral Centre for Sarcoidosis and ILD, Siena University, Siena
| | - Sara Tomassetti
- Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence
| | - Mariaelena Occhipinti
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit no. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Florence
| | - Silvia Bellando Randone
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence
| | - Serena Guiducci
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence
| | - Carlo Vancheri
- Department of Clinical and Experimental Medicine, Regional Referral Centre for Rare Lung Disease, A.O.U. 'Policlinico-Vittorio Emanuele', Catania
| | - Stefano Colagrande
- Department of Experimental and Clinical Biomedical Sciences, Radiodiagnostic Unit no. 2, University of Florence - Azienda Ospedaliero-Universitaria Careggi, Florence
| | - Marco Matucci-Cerinic
- Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence.,Division of Rheumatology, Department of Geriatric Medicine, Azienda Ospedaliero-Universitaria Careggi, Florence, Italy
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Kono M, Nakamura Y, Enomoto Y, Yasui H, Hozumi H, Karayama M, Suzuki Y, Furuhashi K, Miki Y, Hashimoto D, Fujisawa T, Enomoto N, Inui N, Kaida Y, Yokomura K, Koshimizu N, Toyoshima M, Imokawa S, Yamada T, Shirai T, Hayakawa H, Nakamura H, Suda T. Pneumothorax in Patients with Idiopathic Pleuroparenchymal Fibroelastosis: Incidence, Clinical Features, and Risk Factors. Respiration 2021; 100:19-26. [PMID: 33401281 DOI: 10.1159/000511965] [Citation(s) in RCA: 18] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/04/2020] [Accepted: 09/29/2020] [Indexed: 11/19/2022] Open
Abstract
BACKGROUND Idiopathic pleuroparenchymal fibroelastosis (PPFE) is a rare form of idiopathic interstitial pneumonia that is characterized by predominantly upper lobe pleural and subpleural lung parenchymal fibrosis. Pneumothorax is one of the major respiratory complications in PPFE patients; however, its clinical features are poorly understood. OBJECTIVE We aimed to investigate the complication of pneumothorax in patients with idiopathic PPFE. METHODS A retrospective multicenter study involving 89 patients who had been diagnosed with idiopathic PPFE was conducted. We investigated the cumulative incidence, clinical features, and risk factors of pneumothorax after the diagnosis of idiopathic PPFE. RESULTS Pneumothorax developed in 53 patients (59.6%) with 120 events during the observation period (41.8 ± 35.0 months). The cumulative incidence of pneumothorax was 24.8, 44.9, and 53.9% at 1, 2, and 3 years, respectively. Most events of pneumothorax were asymptomatic (n = 85; 70.8%) and small in size (n = 92; 76.7%); 30 patients (56.6%) had recurrent pneumothorax. Chest drainage was required in 23 pneumothorax events (19.2%), and a persistent air leak was observed in 13 (56.5%). Patients with pneumothorax were predominantly male and frequently had pathological diagnoses of PPFE and prior history of pneumothorax and corticosteroid use; they also had significantly poorer survival than those without pneumothorax (log-rank test; p = 0.001). Multivariate analysis revealed that a higher residual volume/total lung capacity ratio was significantly associated with the development of pneumothorax after the diagnosis. CONCLUSION Pneumothorax is often asymptomatic and recurrent in patients with idiopathic PPFE, leading to poor outcomes in some cases.
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Affiliation(s)
- Masato Kono
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan, .,Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan,
| | - Yutaro Nakamura
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Yasunori Enomoto
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Hideki Yasui
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Hironao Hozumi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Masato Karayama
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Yuzo Suzuki
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Kazuki Furuhashi
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Yoshihiro Miki
- Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
| | - Dai Hashimoto
- Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
| | - Tomoyuki Fujisawa
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Noriyuki Enomoto
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Naoki Inui
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
| | - Yusuke Kaida
- Department of Respiratory Medicine, Enshu Hospital, Hamamatsu, Japan
| | - Koshi Yokomura
- Department of Respiratory Medicine, Seirei Mikatahara General Hospital, Hamamatsu, Japan
| | - Naoki Koshimizu
- Department of Respiratory Medicine, Fujieda Municipal General Hospital, Fujieda, Japan
| | - Mikio Toyoshima
- Department of Respiratory Medicine, Hamamatsu Rosai Hospital, Hamamatsu, Japan
| | - Shiro Imokawa
- Department of Respiratory Medicine, Iwata City Hospital, Iwata, Japan
| | - Takashi Yamada
- Department of Respiratory Medicine, Shizuoka City Shizuoka Hospital, Shizuoka, Japan
| | - Toshihiro Shirai
- Department of Respiratory Medicine, Shizuoka General Hospital, Shizuoka, Japan
| | - Hiroshi Hayakawa
- Department of Respiratory Medicine, Tenryu Hospital, National Hospital Organization, Hamamatsu, Japan
| | - Hidenori Nakamura
- Department of Pulmonary Medicine, Seirei Hamamatsu General Hospital, Hamamatsu, Japan
| | - Takafumi Suda
- Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan
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