|
1
|
Ruffer N, Kleefeld F, Holzer MT, Krusche M, Kötter I, Schneider U, Stenzel W. [Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective]. Z Rheumatol 2025; 84:210-218. [PMID: 39316132 PMCID: PMC11965222 DOI: 10.1007/s00393-024-01567-y] [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] [Accepted: 08/07/2024] [Indexed: 09/25/2024]
Abstract
The peripheral nervous system is a classic target organ in systemic vasculitis. In addition, the skeletal muscle can also be affected. Myalgia, muscle weakness and sensory deficits are typical signs, which can lead to severe functional limitations and impaired of quality of life. Vasculitic involvement of the skeletal muscle (vasculitic myopathy [VM]) and peripheral nerves (vasculitic neuropathy [VN]) occurs predominantly in polyarteritis nodosa and small-vessel vasculitis. VM presents with elevated markers of inflammation and is typically characterized by immobilizing myalgia with normal creatine kinase activity and diffuse or patchy areas of hyperintensity on T2-weighted MRI ("MRI myositis without myositis"). In VN, sensor motor deficits predominantly affect the lower extremity in the area supplied by several peripheral nerves (e.g., mononeuritis multiplex) with acute to subacute history. The histopathological examination of nerve and muscle biopsies is the gold standard for the diagnosis of vasculitic manifestations and has a significant impact on the therapeutic approach.
Collapse
Affiliation(s)
- Nikolas Ruffer
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland.
| | - Felix Kleefeld
- Klinik für Neurologie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Marie-Therese Holzer
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
| | - Martin Krusche
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
| | - Ina Kötter
- III. Medizinische Klinik und Poliklinik, Universitätsklinikum Hamburg-Eppendorf, Martinistraße 52, 20246, Hamburg, Deutschland
- Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt, Bad Bramstedt, Deutschland
| | - Udo Schneider
- Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Charité - Universitätsmedizin, Berlin, Deutschland
| | - Werner Stenzel
- Institut für Neuropathologie, Charité - Universitätsmedizin, Berlin, Deutschland
| |
Collapse
|
|
2
|
Iannone C, Pellico MR, Caminati A, Zompatori M, Tescaro L, Luisi F, Elia D, Mirenda MR, Colleoni M, Cassandro R, Harari S, Caporali RF. Anti-neutrophil cytoplasmic antibodies associated interstitial pneumonia: A possible new clinical entity. Eur J Clin Invest 2025:e70025. [PMID: 40108989 DOI: 10.1111/eci.70025] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/04/2024] [Accepted: 02/25/2025] [Indexed: 03/22/2025]
Abstract
BACKGROUND Anti-neutrophil cytoplasmic antibodies (ANCA), a hallmark of systemic vasculitis (SV), have been reported in patients with idiopathic interstitial pneumonia (IIP). However, the clinical significance of ANCA in IIP remains unclear. METHODS We retrospectively studied 101 IP patients diagnosed by pneumologists as idiopathic interstitial pneumonia (IIP,64) and IP with autoimmune features (IPAF,37). ANCA, anti-myeloperoxidase and anti-proteinase-3 were tested by immunofluorescence and ELISA. Chest HRCT patterns, pulmonary function tests (PFTs) and the evolution to SV during a 12-month follow-up were assessed. Multivariable regression analysis was performed to assess the association of baseline covariates with SV. The proximity of patients with close characteristics was investigated by cluster analysis. RESULTS Twenty-one patients (20.8%) were ANCA+, similarly distributed between IPAF and IIP. ANCA+ patients were more likely to have NSIP (p = .02) and bronchiectasis (p = .02) on HRCT, less impaired 6MWD (p = .02), higher CRP (p = .02) and more arthralgias (p < .001) than ANCA- patients. During follow-up, 9 (43%) p-ANCA+ patients, but no ANCA- patients, developed SV (p = .001). p-ANCA+ IP had 26.3 OR (95% CI 3.20-36.8) to evolve to SV within 12 months (p < .0001). Cluster analysis identified one group of 25 patients with significantly higher baseline NSIP (88%), p-ANCA+ (48%), arthralgias (32%), and SV (24%) at 12 months. Nevertheless, 12 p-ANCA+ IP patients never developed SV. CONCLUSIONS ANCA+ IP patients had a high risk of developing SV and need close monitoring and prompt immunotherapy. ANCA+ IP patients not evolving to SV had a diagnosis of IIP or IPAF. These patients need longer observational studies to investigate if they represent a distinct ILD entity.
Collapse
Affiliation(s)
- Claudia Iannone
- ASST G. Pini-CTO. UOC Clinica Reumatologica, Milan, Italy
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
| | - Maria Rosa Pellico
- ASST G. Pini-CTO. UOC Clinica Reumatologica, Milan, Italy
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
| | - Antonella Caminati
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Maurizio Zompatori
- Operative Unit of Radiology, Department of Diagnostic Imaging, MultiMedica IRCCS, Milan, Italy
| | - Lisa Tescaro
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
| | - Francesca Luisi
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Davide Elia
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Maria Rosa Mirenda
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Matteo Colleoni
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
| | - Roberto Cassandro
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Sergio Harari
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
- UO di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria Ed Emodinamica Polmonare, MultiMedica IRCCS, Milan, Italy
| | - Roberto Felice Caporali
- ASST G. Pini-CTO. UOC Clinica Reumatologica, Milan, Italy
- Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
| |
Collapse
|
|
3
|
Zulfiqar F, Bilal M, Shah Y, Morgan S, Fatima R, Singh B, Sebastian SA, Roumia B, Bhatt P, Thallapally VK, Krishnamoorthy G, Hussain SAM. Navigating mesenteric vasculitis: A comprehensive review of literature. Dis Mon 2024; 70:101830. [PMID: 39592294 DOI: 10.1016/j.disamonth.2024.101830] [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: 11/28/2024]
Abstract
Vasculitides are diseases marked by inflammation of the blood vessel walls across various organ systems. The mesenteric vasculitis (MV) affects localized mesenteric vessels of the gastrointestinal tract. It usually occurs as part of a systemic inflammatory process but could also present in isolation. There are very few published reports of isolated mesenteric artery vasculitis. Presenting symptoms often include nausea, vomiting, diarrhea, abdominal pain, rectal bleeding, often complicating the diagnostic process. Diagnosing MV as the cause of abdominal pain can be challenging, and failure to diagnose can result in significant morbidity and mortality. A timely and accurate diagnosis of MV is essential for administering the appropriate immunosuppressive therapy and preventing unnecessary surgical interventions. This review aims to provide a comprehensive discussion of MV, including its clinical presentation, diagnostic approaches, and treatment options, with a focus on achieving early diagnosis to enhance outcomes and prevent complications. Furthermore, this review addresses the diagnostic challenges associated with MV, including the lack of specific criteria and symptom overlap with other gastrointestinal disorders such as atherosclerotic mesenteric ischemia, infections, malignancies, adverse medication effects, and other vessel occlusive processes. It also emphasizes the gaps in current literature regarding optimal diagnostic strategies and the necessity for standardized treatment protocols. By addressing these gaps and challenges, we aim to optimize patient care and improve prognosis for individuals affected by MV.
Collapse
Affiliation(s)
- Fizza Zulfiqar
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | - Muhammad Bilal
- Department of Internal Medicine, Bassett Medical Center, Cooperstown, NY, USA
| | - Yash Shah
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA.
| | - Steele Morgan
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | - Rida Fatima
- Department of Internal Medicine, Bassett Medical Center, Cooperstown, NY, USA
| | - Bryanna Singh
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | | | - Bashar Roumia
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI, USA
| | - Parjanya Bhatt
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | | | - Geetha Krishnamoorthy
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| | - Syed Ali Muttaqi Hussain
- Department of Internal Medicine, Trinity Health Oakland/ Wayne State University, Pontiac, MI, USA
| |
Collapse
|
|
4
|
Mohamed AA, Caussat T, Mouhawasse E, Ali R, Johansen PM, Lucke-Wold B. Neurosurgical Intervention for Nerve and Muscle Biopsies. Diagnostics (Basel) 2024; 14:1169. [PMID: 38893695 PMCID: PMC11172125 DOI: 10.3390/diagnostics14111169] [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: 04/03/2024] [Revised: 05/21/2024] [Accepted: 05/30/2024] [Indexed: 06/21/2024] Open
Abstract
(1) Background: Neurologic and musculoskeletal diseases represent a considerable portion of the underlying etiologies responsible for the widely prevalent symptoms of pain, weakness, numbness, and paresthesia. Because of the subjective and often nonspecific nature of these symptoms, different diagnostic modalities have been explored and utilized. (2) Methods: Literature review. (3) Results: Nerve and muscle biopsy remains the gold standard for diagnosing many of the responsible neurological and musculoskeletal conditions. However, the need for invasive tissue sampling is diminishing as more investigations explore alternative diagnostic modalities. Because of this, it is important to explore the current role of neurosurgical intervention for nerve and muscle biopsies and its current relevance in the diagnostic landscape of neurological and musculoskeletal disorders. With consideration of the role of nerve and muscle biopsy, it is also important to explore innovations and emerging techniques for conducting these procedures. This review explores the indications and emerging techniques for neurological intervention for nerve and muscle biopsies. (4) Conclusions: The role of neurosurgical intervention for nerve and muscle biopsy remains relevant in diagnosing many neurological and musculoskeletal disorders. Biopsy is especially relevant as a supportive point of evidence for diagnosis in atypical cases. Additionally, emerging techniques have been explored to guide diagnostics and biopsy, conduct less invasive biopsies, and reduce risks of worsening neurologic function and other symptoms secondary to biopsy.
Collapse
Affiliation(s)
- Ali A. Mohamed
- Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, USA
| | - Thomas Caussat
- Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, USA
| | - Edwin Mouhawasse
- Charles E. Schmidt College of Medicine, Florida Atlantic University, Boca Raton, FL 33431, USA
| | - Rifa Ali
- College of Medicine, University of Central Florida, Orlando, FL 32827, USA
| | - Phillip M. Johansen
- Department of Neurosurgery and Brain Repair, University of South Florida, Tampa, FL 33613, USA
| | - Brandon Lucke-Wold
- Department of Neurosurgery, University of Florida, Gainesville, FL 32608, USA
| |
Collapse
|
|
5
|
Younger DS. Headaches and Vasculitis. Neurol Clin 2024; 42:389-432. [PMID: 38575258 DOI: 10.1016/j.ncl.2023.12.003] [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: 04/06/2024]
Abstract
Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability making this a disorder of paramount importance to all clinicians. Headache may be an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. Primary neurologic vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible.
Collapse
Affiliation(s)
- David S Younger
- Department of Medicine, Section of Neuroscience, City University of New York School of Medicine, New York, NY, USA; Department of Neurology, White Plains Hospital, White Plains, NY, USA.
| |
Collapse
|
|
6
|
Quiceno JD, Betancur JF, Solano A, Puerta JD, Toro N. A Rare Case of Single-Organ Vasculitis of the Hepatic Artery in a Female Patient: Diagnosis and Management Challenges. Cureus 2024; 16:e58637. [PMID: 38770458 PMCID: PMC11104283 DOI: 10.7759/cureus.58637] [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: 04/20/2024] [Indexed: 05/22/2024] Open
Abstract
This case report describes a rare occurrence of isolated vasculitis of the hepatic artery in a female patient. The patient presented with abdominal pain, fever, and weight loss, and a diagnosis was made through a combination of imaging studies and serological evaluation of systemic vasculitis. The management of this case was challenging because of the involvement of the hepatic artery without any other clinical manifestations of the systemic disease, apart from the presence of rheumatoid factor and anti-citrullinated cyclic peptide. The authors highlight the importance of considering vasculitis as a potential diagnosis in patients with unexplained abdominal pain and fever and the need for a multidisciplinary approach to the management of these patients. This case also emphasizes the potential complications of vasculitis, including aneurysm formation, and the need for close monitoring and follow-up of these patients.
Collapse
Affiliation(s)
- Julian D Quiceno
- Internal Medicine, Clínica Medellín Grupo QuirónSalud, Medellin, COL
| | - Juan F Betancur
- Internal Medicine, Clínica Medellín Grupo QuirónSalud, Medellin, COL
- Internal Medicine, Internal Medicine Investigation Unit, Centro de Investigaciones Clínicas SURA, Medellin, COL
| | | | - Jose D Puerta
- Vascular Medicine, Universidad de Antioquia, Medellin, COL
| | - Nancy Toro
- Radiology, Clínica Medellín Grupo QuirónSalud, Medellín, COL
| |
Collapse
|
|
7
|
Younger DS. Systemic vasculitis and headache. Curr Opin Neurol 2023; 36:631-646. [PMID: 37865837 PMCID: PMC10624412 DOI: 10.1097/wco.0000000000001223] [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: 10/23/2023]
Abstract
PURPOSE OF REVIEW Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system or so called neurovasculitides, lead to pervasive injury and disability making these disorder of paramount importance to clinicians. RECENT FINDINGS Headache is an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary adult and pediatric CNS vasculitides predicated on achievements in primary systemic forms. SUMMARY Vasculitis can be diagnosed with certainty after intensive evaluation that includes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, tempered by the recognition of anticipated medication side effects.
Collapse
Affiliation(s)
- David S Younger
- Department of Medicine, Section of Neuroscience, City University of New York School of Medicine, New York, NY; Department of Neurology, White Plains Hospital, White Plains, New York, USA
| |
Collapse
|
|
8
|
Deniz R, Güzelbey T, Özgür DS, Karaalioğlu B, Akkuzu G, Yıldırım F, Bes C. Isolated inferior thyroidal artery vasculitis: A rare cause of neck pain. Int J Rheum Dis 2023; 26:2294-2296. [PMID: 37191117 DOI: 10.1111/1756-185x.14733] [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: 03/09/2023] [Accepted: 05/05/2023] [Indexed: 05/17/2023]
Abstract
Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non-invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.
Collapse
Affiliation(s)
- Rabia Deniz
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Tevfik Güzelbey
- Department of Radiology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Duygu Sevinç Özgür
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Bilgin Karaalioğlu
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Gamze Akkuzu
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Fatih Yıldırım
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| | - Cemal Bes
- Department of Rheumatology, University of Health Sciences Başakşehir Çam and Sakura City Hospital, Istanbul, Turkey
| |
Collapse
|
|
9
|
Liu L, Li J, Gan T, Yang Y, Tian X. Isolated Coronary Arteritis in Adults: a Single-Center Experience from China. J Cardiovasc Transl Res 2023; 16:1184-1193. [PMID: 37097590 DOI: 10.1007/s12265-023-10388-4] [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: 05/28/2022] [Accepted: 04/14/2023] [Indexed: 04/26/2023]
Abstract
Isolated coronary arteritis (ICA) is an extremely rare and life-threatening vasculitis with only a limited number of reports in the literature. We retrospectively reviewed the clinical data of 10 ICA patients in our center from 2012 to 2022 and compared them with patients with Takayasu arteritis who presented with coronary arteritis initially (TAK-CA patients). We found that ICA predominantly affected women and most commonly involved the ostium and the proximal segment of the coronary arteries, causing mainly stenotic lesions. The C-reactive protein and erythrocyte sedimentation rate were grossly normal and significantly lower than those of TAK-CA patients (p = 0.027, p = 0.009, respectively). Intravascular ultrasound imaging showed superiority in differentiating coronary vasculitis from atherosclerosis. Restenosis of the coronary arteries occurred rapidly if not treated promptly and appropriately. Systemic glucocorticoid combined with immunosuppressive agents, especially cyclophosphamide, was a promising strategy for treating ICA.
Collapse
Affiliation(s)
- Lingyu Liu
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Beijing, 100730, China
| | - Jing Li
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Beijing, 100730, China
| | - Tianpeng Gan
- Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China
| | - Yunjiao Yang
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Beijing, 100730, China
| | - Xinping Tian
- Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, No. 1 Shuaifuyuan, Beijing, 100730, China.
- National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Ministry of Science & Technology, Beijing, China.
- State Key Laboratory of Complex Severe and Rare Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College Hospital (PUMCH), Beijing, 100730, China.
| |
Collapse
|
|
10
|
Kaviani R, Farrell J, Dehghan N, Moosavi S. Single organ hepatic artery vasculitis as an unusual cause of epigastric pain: A case report. World J Clin Cases 2022; 10:9384-9389. [PMID: 36159425 PMCID: PMC9477682 DOI: 10.12998/wjcc.v10.i26.9384] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2022] [Revised: 07/07/2022] [Accepted: 07/31/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Single-organ vasculitis (SOV) is characterized by inflammation of a blood vessel, affecting one organ, such as the skin, genitourinary system, or the aorta without systemic features. Gastrointestinal SOV is rare, with hepatic artery involvement reported only in two prior published cases. Herein, we presented a case of isolated hepatic artery vasculitis presenting after Pfizer-BioNTech mRNA corona virus disease 2019 (COVID-19) vaccination.
CASE SUMMARY A 50-year-old woman with hypertension presented to our Emergency Department with recurrent diffuse abdominal pain that localized to the epigastrium and emesis without diarrhea that began eight days after the second dose of the Pfizer-BioNTech COVID-19 vaccine. Blood work revealed an elevated C-reactive protein (CRP) of 19 mg/L (normal < 4.8 mg/L), alkaline phosphatase 150 U/L (normal 25-105 U/L), gamma-glutamyl transferase (GGT) 45 U/L (normal < 43 U/L) and elevated immunoglobulins (Ig) G 18.4 g/L (normal 7-16 g/L) and IgA 4.4 g/L (normal 0.7-4 g/L). An abdominal computed tomography revealed findings in keeping with hepatic artery vasculitis. A detailed review of her history and examination did not reveal infectious or systemic autoimmune causes of her presentation. An extensive autoimmune panel was unremarkable. COVID-19 polymerase chain reaction nasopharyngeal swab, human immunodeficiency virus, viral hepatitis and Heliobacter pylori serology were negative. At six months, the patient’s symptoms, and blood work spontaneously normalized.
CONCLUSION High clinical suspicion of SOV is required for diagnosis in patients with acute abdominal pain and dyspepsia.
Collapse
Affiliation(s)
- Rojin Kaviani
- Internal Medicine, University of British Columbia, Vancouver V5Z 1M9, British Columbia, Canada
| | - Jessica Farrell
- Division of Radiology, Providence Health Care, Vancouver V5T 3N4, British Columbia, Canada
| | - Natasha Dehghan
- Division of Rheumatology, Providence Health Care, Vancouver V5T 3N4, British Columbia, Canada
| | - Sarvee Moosavi
- Division of Gastroenterology, University of British Columbia, Vancouver V6Z 2K5, British Columbia, Canada
| |
Collapse
|
|
11
|
Abstract
PURPOSE OF REVIEW To summarize the histologic findings of vasculitis, and to give some practical considerations on biopsy samples. RECENT FINDINGS The larger use of imaging and the discoveries of serological markers in the diagnosis of vasculitis have increased the clinical recognition of these entities. Nevertheless, biopsy remains the gold standard for diagnosis in most cases. So far, biopsies are also useful to obtain information about prognosis and to guide a more specific treatment. In recent years, less invasive diagnostic approaches have become available, lowering the risks related to the procedure and permitting a definite diagnosis in most cases. Histological examination permits a definite diagnosis of vasculitis. However, the findings may be nonspecific if not evaluated in the proper clinical setting. The interaction between clinicians and pathologists is crucial to obtain a definite diagnosis.
Collapse
|
|
12
|
Yanartaş M, Karakoç AZ, Zengin A, Taş S, Olgun Yildizeli Ş, Mutlu BL, Ataş H, Alibaz-Öner F, Inanç N, Direskeneli H, Bozkurtlar E, Erkilinç A, Çimşit Ç, Bekiroğlu GN, Yildizeli B. Multimodal Approach of Isolated Pulmonary Vasculitis: A Single-Institution Experience. Ann Thorac Surg 2021; 114:1253-1261. [PMID: 34506746 DOI: 10.1016/j.athoracsur.2021.08.009] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2021] [Revised: 07/11/2021] [Accepted: 08/02/2021] [Indexed: 11/01/2022]
Abstract
BACKGROUND Isolated pulmonary vasculitis (IPV) is a single-organ vasculitis of unknown etiology and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to review our clinical experience with pulmonary endarterectomy in patients with CTEPH secondary to IPV. METHODS Data were collected prospectively for consecutive patients who underwent pulmonary endarterectomy and had a diagnosis of IPV at or after surgery. RESULTS We identified nine patients (six female, median age 48 (23-55) years) with IPV. The diagnosis was confirmed after histopathological examination of all surgical materials. The mean duration of disease before surgery was 88.0 ±70.2 months. Exercise-induced dyspnea was the presenting symptom in all patients. Pulmonary endarterectomy was bilateral in six patients and unilateral in three. No mortality was observed, however, one patient had pulmonary artery stenosis and stent implantation was performed. All patients received immunosuppressive therapies after surgery. Mean pulmonary artery pressure decreased significantly from 30(19-67) mm Hg to 21(15-49) mm Hg after surgery (p <0.05). Pulmonary vascular resistance also improved significantly from 270 (160-1600) to 153 (94-548) dyn/s/cm-5 (p<0.05). After a median follow-up of 41 months, all but one patient had improved to the New York Heart Association functional class I. CONCLUSIONS Isolated pulmonary vasculitis can mimic CTEPH, and these patients can be diagnosed with pulmonary endarterectomy. Furthermore, surgery has not only diagnostic but also therapeutic value for IPV when stenotic and/or thrombotic lesions are surgically accessible. A multidisciplinary experienced CTEPH team is critical for management of these unique patients.
Collapse
Affiliation(s)
- Mehmed Yanartaş
- University of Health Sciences,Kartal Koşuyolu Teaching and Education Hospital,Department of Cardiovascular Surgery, Istanbul.
| | - Ayşe Zehra Karakoç
- University of Health Sciences,Kartal Koşuyolu Teaching and Education Hospital,Department of Cardiovascular Surgery, Istanbul
| | - Ahmet Zengin
- University of Health Sciences,Kartal Koşuyolu Teaching and Education Hospital,Department of Cardiovascular Surgery, Istanbul
| | - Serpil Taş
- University of Health Sciences,Kartal Koşuyolu Teaching and Education Hospital,Department of Cardiovascular Surgery, Istanbul
| | - Şehnaz Olgun Yildizeli
- Marmara University School of Medicine,Department of Department of Pulmonology and Intensive Care, Istanbul
| | - Bu Lent Mutlu
- Marmara University School of Medicine,Department of Cardiology, Istanbul
| | - Halil Ataş
- Marmara University School of Medicine,Department of Cardiology, Istanbul
| | - Fatma Alibaz-Öner
- Marmara University School of Medicine,Department of Internal Medicine,Division of Rheumatology, Istanbul
| | - Nevsun Inanç
- Marmara University School of Medicine,Department of Internal Medicine,Division of Rheumatology, Istanbul
| | - Haner Direskeneli
- Marmara University School of Medicine,Department of Internal Medicine,Division of Rheumatology, Istanbul
| | - Emine Bozkurtlar
- Marmara University School of Medicine,Department of Pathology, Istanbul
| | - Atakan Erkilinç
- University of Health Sciences,Kartal Koşuyolu Teaching and Education Hospital,Department of Anesthesia, Istanbul
| | - Çagatay Çimşit
- Marmara University School of Medicine,Department of Radiology, Istanbul
| | - G Nural Bekiroğlu
- Marmara University School of Medicine, Department of Biostatistics, Istanbul, Turkey
| | - Bedrettin Yildizeli
- Marmara University School of Medicine,Department of Thoracic Surgery, Istanbul
| |
Collapse
|
|
13
|
Smith SF, Miakhil SI, Lockett C. An unusual cause of testicular pain: single organ lymphocytic vasculitis. JOURNAL OF CLINICAL UROLOGY 2021. [DOI: 10.1177/2051415818824777] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Affiliation(s)
| | | | - Chris Lockett
- Department of Urology, Peterborough City Hospital, UK
| |
Collapse
|
|
14
|
Martins-Martinho J, Dourado E, Khmelinskii N, Espinosa P, Ponte C. Localized Forms of Vasculitis. Curr Rheumatol Rep 2021; 23:49. [PMID: 34196889 PMCID: PMC8247627 DOI: 10.1007/s11926-021-01012-y] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/21/2021] [Indexed: 12/26/2022]
Abstract
Purpose of Review To provide an updated review on epidemiology, clinical manifestations, diagnostic assessment, treatment, and prognosis of localized vasculitis, following the 2012 Revised International Chapel Hill Consensus Conference Nomenclature on single-organ vasculitis. Recent Findings Localized, single-organ vasculitides encompass a group of rare conditions in which there is no evidence of concomitant systemic vasculitis. Most data on this topic derives from case reports and small case series. Although some aspects of these diseases, such as clinical manifestations and histologic findings, have already been extensively investigated, there is still a lack of robust data concerning the pathogenesis, epidemiology, and treatment. Summary Localized vasculitides may have a wide range of clinical features depending on the organ affected. The inflammatory process may have a multifocal/diffuse or unifocal distribution. Diagnosis is usually based on histopathology findings and exclusion of systemic vasculitis, which may frequently pose a challenge. Further research on treatment is warranted.
Collapse
Affiliation(s)
- Joana Martins-Martinho
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal. .,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal.
| | - Eduardo Dourado
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Nikita Khmelinskii
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal
| | - Pablo Espinosa
- Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal
| | - Cristina Ponte
- Rheumatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal.,Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte, 1649-035, Lisbon, Portugal
| |
Collapse
|
|
15
|
dell'Omo R, Filippelli M, De Turris S, Cimino L, Steel DH, Pavesio CE, Govetto A, Chehaibou I, Parmeggiani F, Romano MR, Ziccardi L, Pirozzi E, Costagliola C. Fluorescein Angiography Findings in Eyes With Lamellar Macular Hole and Epiretinal Membrane Foveoschisis. Invest Ophthalmol Vis Sci 2021; 62:34. [PMID: 33512403 PMCID: PMC7846948 DOI: 10.1167/iovs.62.1.34] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
Purpose The purpose of this paper was to study fluorescein angiography (FA) findings in eyes with lamellar macular hole (LMH), and epiretinal membrane (ERM) foveoschisis. Methods In this prospective, observational case series, 46 eyes of patients affected by either LMH or ERM foveoschisis were examined using optical coherence tomography (OCT) and FA. All patients underwent a comprehensive ophthalmological examination and a general workup to exclude uveitis. Main outcome measures were: presence of FA abnormalities, measurements of the areas of vascular leakage, and intensity of pixels in the vitreous. Results Twenty-four (52.2%) eyes with LMH and 22 (47.8%) with ERM foveoschisis were studied. Overall, FA abnormalities were found in 20 (83.3%) eyes with LMH and 18 (81.8%) with ERM foveoschisis. The median areas of posterior pole and peripheral leakage were 7.52 vs. 1.07 mm2 (P = 0.03) and 21.8 vs. 3.74 mm2 (P = 0.02) in the LMH and ERM foveoschisis group, respectively. Disk hyperfluorescence was found in 8 and 4 eyes and perivascular leak in 10 and 4 eyes with LMH and ERM foveoschisis, respectively. OCT-derived measurements of vitreous intensity did not differ between the two groups, and the investigational workup for uveitis was negative in all patients. Conclusions Discrete areas of central and peripheral leakage are commonly found in eyes with LMH and ERM foveoschisis, whereas perivascular leak and hyperfluorescence of the disc are less frequently observed. These findings suggest that breakdown of the retinal blood barrier, involving the posterior pole and the periphery, is frequently associated with these two vitreoretinal disorders.
Collapse
Affiliation(s)
- Roberto dell'Omo
- Department of Medicine and Health Sciences "Vincenzo Tiberio," University of Molise, Campobasso, Italy
| | - Mariaelena Filippelli
- Department of Medicine and Health Sciences "Vincenzo Tiberio," University of Molise, Campobasso, Italy
| | | | - Luca Cimino
- Ocular Immunology Unit, Azienda USL-IRCCS of Reggio Emilia, Reggio Emilia, Italy
| | - David H Steel
- Department of Ophthalmology, Sunderland Eye Infirmary, Sunderland, United Kingdom and Newcastle University, Sunderland, Newcastle, United Kingdom
| | | | - Andrea Govetto
- Fatebenefratelli-Oftalmico Hospital, ASST-Fatebenefratelli-Sacco, Milan, Italy
| | - Ismael Chehaibou
- Université de Paris, Ophthalmology Department, AP-HP, Hôpital Lariboisière, Paris, France
| | - Francesco Parmeggiani
- Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara, Italy
| | - Mario R Romano
- Department of Biomedical Science, Humanitas University, Pieve Emanuele, Milan, Italy
| | - Lucia Ziccardi
- Neurophysiology and Neurophthalmology Unit, IRCCS- Fondazione Bietti, Rome, Italy
| | - Enza Pirozzi
- Institute of Ophthalmology, Santa Croce e Carle Hospital, Cuneo, Italy
| | - Ciro Costagliola
- Department of Medicine and Health Sciences "Vincenzo Tiberio," University of Molise, Campobasso, Italy
| |
Collapse
|
|
16
|
Aghayev A, Steigner ML. Systemic vasculitides and the role of multitechnique imaging in the diagnosis. Clin Radiol 2021; 76:488-501. [PMID: 33812649 DOI: 10.1016/j.crad.2021.03.002] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2020] [Accepted: 03/02/2021] [Indexed: 11/17/2022]
Abstract
Vasculitis, a systemic disease characterised by inflammation of the blood vessels, remains challenging to diagnose and manage. Vessel size has been the basis for classifying systemic vasculitides. Imaging plays a vital role in diagnosing this challenging disease. This review article aims (a) to summarise up-to-date literature in this field, as well as include classification updates and (b) to review available imaging techniques, recent advances, and emphasis on imaging findings to diagnose large vessel vasculitides.
Collapse
Affiliation(s)
- A Aghayev
- Cardiovascular Imaging Program, Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
| | - M L Steigner
- Cardiovascular Imaging Program, Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| |
Collapse
|
|
17
|
Harada T, Sasaki Y, Tokunaga T, Yoshizawa A, Miura S, Ikeda K, Saito T, Hiroshige J. 18F-Fluorodeoxyglucose positron emission tomography computed tomography detection of single organ vasculitis of the breast: A case report. Medicine (Baltimore) 2021; 100:e25259. [PMID: 33761723 PMCID: PMC9281965 DOI: 10.1097/md.0000000000025259] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/19/2020] [Accepted: 03/04/2021] [Indexed: 01/05/2023] Open
Abstract
RATIONALE Although single organ vasculitis (SOV) is a rare occurrence and it is difficult to diagnose, its possibility as a cause of fever of unknown origin (FUO) must be considered. Recently, the usefulness of 18F-fluorodeoxyglucose positron emission tomography computed tomography (FDG PET/CT) in the diagnosis of unknown fevers due to vasculitis, especially in cases of small and medium-sized vasculitis, has begun to be pointed out. PATIENT CONCERNS We report the case of an 84-year-old woman with persisting fever for more than 2 weeks. She had no accompanying symptoms, other than fever, and the physical examination, echocardiography, and contrast-enhanced CT did not reveal any diagnostic clue. DIAGNOSES The FDG PET/CT revealed positive uptakes of FDG in the left breast, with a standardized uptake value (SUV) of 2.9. The biopsy specimen of the left breast lesion revealed rupture of the elastic plate and evidence of fibrinoid necrosis of arteries, leading to the diagnosis of polyarteritis (PAN). Further angiographic examination and additional imaging did not reveal the presence of other lesions. Therefore, the diagnosis was established as a PAN-SOV of the left breast. INTERVENTIONS This patient has improved with follow-up only. OUTCOMES There has been no evidence of a relapse of PAN over a 5-year follow-up period. LESSONS SOV presenting with unspecific local symptoms is difficult to diagnose based on the medical history and clinical examination. Our findings show that early "Combination of PET-CT and biopsy" can be a powerful diagnostic tool in patients with FUO for whom diagnosis of the underlying cause is difficult despite appropriate clinical examination.
Collapse
Affiliation(s)
- Taku Harada
- Division of General Medicine, Showa University Koto Toyosu Hospital, Tokyo
- Division of Diagnostic and Generalist Medicine, Dokkyo Medical University Hospital, Tochigi
| | - Yosuke Sasaki
- Department of General Medicine and Emergency Care, Toho University School of Medicine
| | | | - Ayuha Yoshizawa
- Department of Breast Surgery, Showa University Koto Toyosu Hospital
| | - Sakiko Miura
- Department of Diagnostic Pathology, NTT Medical Center
- Department of Pathology, Showa University School of Medicine, Tokyo
| | - Keiichiro Ikeda
- Division of General Medicine, Showa University Koto Toyosu Hospital, Tokyo
| | - Tsukasa Saito
- Department of Internal Medicine, Saitama Shinrin Hospital, Saitama, Japan
| | - Juichi Hiroshige
- Division of General Medicine, Showa University Koto Toyosu Hospital, Tokyo
| |
Collapse
|
|
18
|
González-Meléndez A, Medina-Parrilla EJ, Vélez R, Vilá LM. Isolated intestinal polyarteritis nodosa in an elderly patient. BMJ Case Rep 2021; 14:e241431. [PMID: 33664043 PMCID: PMC7934761 DOI: 10.1136/bcr-2020-241431] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/12/2021] [Indexed: 11/04/2022] Open
Abstract
Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.
Collapse
Affiliation(s)
- Ariana González-Meléndez
- Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
| | - Eduardo J Medina-Parrilla
- Department of Pathology and Laboratory Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
| | - Román Vélez
- Department of Pathology and Laboratory Medicine, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
| | - Luis M Vilá
- Department of Medicine, Division of Rheumatology, University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico
| |
Collapse
|
|
19
|
Hočevar A, Tomšič M, Perdan Pirkmajer K. Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa. Curr Rheumatol Rep 2021; 23:14. [PMID: 33569653 DOI: 10.1007/s11926-021-00983-2] [Citation(s) in RCA: 28] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/05/2021] [Indexed: 01/05/2023]
Abstract
PURPOSE OF THE REVIEW Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries. Different etiopathogenetic and clinical variants of the disease have been recognized over the past decades. In the present paper, we review the clinical features, diagnosis, and treatment of the different subtypes of the disease. RECENT FINDINGS The diagnosis of polyarteritis nodosa is primarily based on clinical findings, imaging, and histopathological investigations. Microbiological and genetic investigations complement the diagnostic work-up. Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange. PAN is a disease with heterogeneous presentations, severity, and therapeutic approaches. The overall prognosis of this disease is improving, mainly due to early diagnosis and more effective treatments. Treatment choices are guided mainly by the disease subtype and severity. In this review, we have presented the current knowledge on PAN clinical variants, their classification, diagnosis, and treatment approaches.
Collapse
Affiliation(s)
- Alojzija Hočevar
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta, 62 1000, Ljubljana, Slovenia. .,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
| | - Matija Tomšič
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta, 62 1000, Ljubljana, Slovenia.,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Katja Perdan Pirkmajer
- Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta, 62 1000, Ljubljana, Slovenia.,Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| |
Collapse
|
|
20
|
Lin YC, Kalot MA, Husainat NM, Byram K, Dua AB, James KE, Springer JM, Turgunbaev M, Villa-Forte A, Abril A, Langford C, Maz M, Chung SA, Mustafa RA. Polyarteritis Nodosa: A Systematic Review of Test Accuracy and Benefits and Harms of Common Treatments. ACR Open Rheumatol 2021; 3:91-100. [PMID: 33512781 PMCID: PMC7882539 DOI: 10.1002/acr2.11189] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/28/2020] [Accepted: 09/28/2020] [Indexed: 11/08/2022] Open
Abstract
OBJECTIVE The object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with primary systemic polyarteritis nodosa (PAN). METHODS A systematic search of published English-language literature was performed in Ovid Medline, PubMed, Embase, and the Cochrane Library from the inception of each database through August 2019. Articles were screened for suitability in addressing patient, intervention, comparison, and outcome questions, with studies presenting the highest level of evidence given preference. RESULTS Of 137 articles selected for data abstraction, we analyzed 21 observational studies and seven randomized controlled trials (RCTs). The results showed indirect evidence that a deep skin biopsy provides good diagnostic accuracy. A combined nerve and muscle biopsy should be obtained for patients with PAN with peripheral neuropathy. Cyclophosphamide with high-dose glucocorticoids (GCs) is effective as an induction treatment for newly diagnosed active and severe PAN. GC monotherapy is adequate in the majority of patients with nonsevere PAN, although it has a high relapse rate with GC taper. There was insufficient data in determining the optimal duration of non-GC and GC maintenance therapy. Tumor necrosis factor inhibitors are effective treatment for patients with deficiency of adenosine deaminase 2 (DADA2) with stroke and vasculitis manifestations. CONCLUSION This comprehensive systematic review synthesizes and evaluates the harms and benefits of different treatment options and the accuracy of commonly used tests for the diagnosis of systemic PAN. Data for diagnosis and management of PAN and DADA2 are mostly limited to observational studies. More high-quality RCTs are needed.
Collapse
Affiliation(s)
- Yih Chang Lin
- University of South Florida, Tampa, Florida, United States
| | - Mohamad A Kalot
- The State University of New York at Buffalo, New York, United States
| | | | - Kevin Byram
- Vanderbilt University Medical Center, Nashville, Tennessee, United States
| | - Anisha B Dua
- Northwestern University Feinberg School of Medicine, Chicago, Illinois, United States
| | - Karen E James
- University of Utah Health, Salt Lake City, United States
| | - Jason M Springer
- Vanderbilt University Medical Center, Nashville, Tennessee, United States
| | - Marat Turgunbaev
- American College of Rheumatology, Atlanta, Georgia, United States
| | | | - Andy Abril
- Mayo Clinic, Jacksonville, Florida, United States
| | | | - Mehrdad Maz
- University of Kansas Medical Center, Kansas City, United States
| | - Sharon A Chung
- University of California, San Francisco Medical Center, California, United States
| | - Reem A Mustafa
- University of Kansas Medical Center, Kansas City, United States.,Department of Health Research Methods, Evidence and Impact, McMaster University, Hamilton, Canada
| |
Collapse
|
|
21
|
Clifford AH, Arafat A, Idrees JJ, Roselli EE, Tan CD, Rodriguez ER, Svensson LG, Blackstone E, Johnston D, Pettersson G, Soltesz E, Hoffman GS. Outcomes Among 196 Patients With Noninfectious Proximal Aortitis. Arthritis Rheumatol 2019; 71:2112-2120. [DOI: 10.1002/art.40855] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/04/2017] [Accepted: 11/27/2018] [Indexed: 12/17/2022]
Affiliation(s)
- Alison H. Clifford
- Cleveland Clinic Foundation, Cleveland, Ohio, and University of Alberta Edmonton Alberta Canada
| | - Amr Arafat
- Cleveland Clinic Foundation, Cleveland, Ohio, and Tanta University Tanta Egypt
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
22
|
Takase Y, Murakami Y, Nishi J, Tokunaga O, Matsumoto T, Aishima S. A unique autopsy case of ascending aortic dissection caused by giant cell arteritis without drug therapy. Pathol Int 2019; 69:614-618. [DOI: 10.1111/pin.12845] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2019] [Accepted: 07/13/2019] [Indexed: 02/07/2023]
Affiliation(s)
- Yukari Takase
- Departments of Pathology & Microbiology, Faculty of MedicineSaga University Saga Japan
| | | | - Jumpei Nishi
- Department of Emergency MedicineFaculty of MedicineSaga University Saga Japan
- Division of Dermatology, Department of Internal MedicineFaculty of MedicineSaga University Saga Japan
| | - Osamu Tokunaga
- Department of Diagnostic PathologyShonan Fujisawa Tokushukai Hospital Kanagawa Japan
| | - Toshiharu Matsumoto
- Department of Diagnostic PathologyJuntendo University Nerima Hospital Tokyo Japan
| | - Shinichi Aishima
- Departments of Pathology & Microbiology, Faculty of MedicineSaga University Saga Japan
| |
Collapse
|
|
23
|
Stewart M, Marcotte G, Seidman MA, Dehghan N. Polyarteritis nodosa isolated to the testis and urinary bladder in the setting of cryptorchidism: a case report and literature review. J Med Case Rep 2019; 13:236. [PMID: 31362782 PMCID: PMC6668096 DOI: 10.1186/s13256-019-2172-y] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2019] [Accepted: 06/21/2019] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Polyarteritis nodosa is a small vessel to medium vessel vasculitis that frequently presents with multi-organ involvement, but can sometimes be limited to single organs such as the testes. Patients often require treatment with glucocorticoids, plus or minus additional immunosuppressive therapy depending on the severity of the disease. We describe a rare case of polyarteritis nodosa involving the right testis and urinary bladder without other systemic features of vasculitis. CASE PRESENTATION A previously healthy 54-year-old First Nations Canadian man presented with intermittent gross hematuria. He underwent surgical excision of his right testis for cryptorchidism and a transurethral resection of a bladder mass. Histology showed an active medium vessel vasculitis in both organs. On extensive clinical, laboratory, and radiographic review, he had no systemic features of vasculitis. On 2-year follow-up, he has not required any systemic therapy and has not developed further symptoms. CONCLUSION Single organ polyarteritis nodosa can sometimes be managed with surgical excision of the involved organ alone. Although our patient had two organs involved, we extrapolated the results of our literature search to guide his care. This case highlights the potential for surgical excision to cure polyarteritis nodosa despite the involvement of two organs in the absence of symptoms and signs of systemic vasculitis.
Collapse
Affiliation(s)
- Mohan Stewart
- Department of Medicine, University of British Columbia, Vancouver, Canada.
| | - Greg Marcotte
- Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada
| | - Michael A Seidman
- Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada.,Department of Pathology & Laboratory Medicine, University of British Columbia, Vancouver, Canada
| | - Natasha Dehghan
- Division of Rheumatology, Department of Medicine, University of British Columbia, Vancouver, Canada
| |
Collapse
|
|
24
|
Suo L, Perez LC, Finch CJ. Testicular granulomatous vasculitis mimicking testicular torsion in an anti-neutrophil cytoplasmic antibody-associated vasculitis patient. SAGE Open Med Case Rep 2019; 7:2050313X18823451. [PMID: 30719308 PMCID: PMC6341501 DOI: 10.1177/2050313x18823451] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2018] [Accepted: 12/14/2018] [Indexed: 12/04/2022] Open
Abstract
Testicular vasculitis is uncommon and can be easily misdiagnosed. Early identification and treatment are essential for patient care. A 63-year-old man, presenting with testicular pain and swelling, had been treated with antibiotics for weeks. A month later, he developed severe left testicular pain and systemic symptoms. Scrotal ultrasound was suggestive of testicular torsion due to no blood flow. Pathology of the orchiectomy specimen demonstrated testicular granulomatous vasculitis involving small- to medium-sized arteries. Additional work-up of blood tests contained positive antinuclear, anti-proteinase 3 and anti-myeloperoxidase antibodies. Erythrocyte sedimentation rate and C-reactive protein were also elevated. Diagnosis of anti-neutrophil cytoplasmic antibody–associated vasculitis was made and prednisone was started. During more than 1-year follow-up, the patient’s systemic symptoms were resolving gradually with no involvement of the other testis.
Collapse
Affiliation(s)
- Liye Suo
- Department of Pathology & Immunology, Baylor College of Medicine, Houston, TX, USA
| | - Luis Carlos Perez
- Department of Pathology & Immunology, Baylor College of Medicine, Houston, TX, USA
| | - Christie Jean Finch
- Department of Pathology & Immunology, Baylor College of Medicine, Houston, TX, USA
| |
Collapse
|
|
25
|
Sheemar A, Takkar B, Temkar S, Sood R, Chawla R, Venkatesh P. Retinal Vasculitis Is Principally a Single-Organ Vasculitis of the Eye. J Clin Rheumatol 2018; 24:87-89. [DOI: 10.1097/rhu.0000000000000671] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Affiliation(s)
- Abhishek Sheemar
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Brijesh Takkar
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Shreyas Temkar
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Rita Sood
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Rohan Chawla
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| | - Pradeep Venkatesh
- From the *Retina and Uvea Services, Dr R. P. Centre for Ophthalmic Sciences, and †Department of Medicine, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
| |
Collapse
|
|
26
|
Bhatia S, Herrera Hernandez LP, Kamboj AK, Rieck KM. Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling. Am J Med 2018; 131:e55-e56. [PMID: 28943380 DOI: 10.1016/j.amjmed.2017.08.040] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/18/2017] [Revised: 08/24/2017] [Accepted: 08/24/2017] [Indexed: 10/18/2022]
Affiliation(s)
- Subir Bhatia
- Department of Internal Medicine, Mayo Clinic, Rochester, Minn
| | | | - Amrit K Kamboj
- Department of Internal Medicine, Mayo Clinic, Rochester, Minn
| | - Katie M Rieck
- Division of Hospital Internal Medicine, Mayo Clinic, Rochester, Minn.
| |
Collapse
|
|
27
|
Sag E, Batu ED, Ozen S. Childhood systemic vasculitis. Best Pract Res Clin Rheumatol 2017; 31:558-575. [PMID: 29773273 DOI: 10.1016/j.berh.2017.11.009] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2017] [Revised: 11/16/2017] [Accepted: 11/20/2017] [Indexed: 01/19/2023]
Abstract
Vasculitides are characterized by inflammation of the vessel wall. Most of the vasculitides tend to occur in vessels of a specific size and certain target organs. In this review, we discuss each specific childhood vasculitis according to the latest Chapel Hill Consensus Conference 2012 nomenclature system and the Ankara 2008 classification criteria. We have also reviewed the clinical and laboratory characteristics and the recent treatment recommendations for the vasculitides we encounter in children.
Collapse
Affiliation(s)
- Erdal Sag
- Hacettepe University, Ihsan Dogramaci Children's Hospital, Department of Pediatric Rheumatology, Turkey
| | - Ezgi Deniz Batu
- Hacettepe University, Ihsan Dogramaci Children's Hospital, Department of Pediatric Rheumatology, Turkey
| | - Seza Ozen
- Hacettepe University, Ihsan Dogramaci Children's Hospital, Department of Pediatric Rheumatology, Turkey.
| |
Collapse
|
|
28
|
Abstract
Systemic vasculitides are caused by inflammation of blood vessels and can affect any organ and any part of the gastrointestinal tract, hepatic and biliary system, as well as the pancreas. These disorders can cause a wide array of gastrointestinal manifestations, from asymptomatic elevated transaminase levels and mild abdominal pain to potentially life-threatening bowel perforations and peritonitis. A diagnosis based solely on gastrointestinal symptoms is challenging as these manifestations are not specific. Conversely, diagnostic and therapeutic delays can be rapidly detrimental. In this article, we review the epidemiology, characteristics and management of the main gastrointestinal manifestations of systemic vasculitides, including polyarteritis nodosa and antineutrophil cytoplasm antibody-associated vasculitides, as well as isolated vasculitides limited to the gastrointestinal tract.
Collapse
|
|
29
|
Pelegrín L, Hernández-Rodríguez J, Espinosa G, Llorenç V, Sainz-de-la-Maza M, Fontenla JR, Martínez JA, Cid MC, Adán A. Characterization of isolated retinal vasculitis. Analysis of a cohort from a single center and literature review. Autoimmun Rev 2017; 16:237-243. [PMID: 28137481 DOI: 10.1016/j.autrev.2017.01.006] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/24/2016] [Accepted: 11/06/2016] [Indexed: 02/09/2023]
Abstract
INTRODUCTION Isolated retinal vasculitis (IRV) is an inflammatory condition of unknown etiology confined to the retinal vessels. In contrast to secondary retinal vasculitis (RV), IRV has not been well characterized. OBJECTIVE To describe and characterize isolated forms of RV. METHODS We performed a retrospective review (2006-2016) of IRV patients from a multidisciplinary Uveitis Unit. RV diagnosis was based on funduscopic and fluorescein angiography findings. To distinguish between secondary RV and IRV, evaluations included clinical assessment, several inflammatory, autoimmune and microbiological laboratory markers, and a chest radiography. Ophthalmological features at disease onset, therapeutic interventions, ocular relapses, visual outcomes and laboratory findings were recorded. Our cases were subsequently compared with those from a literature review. RESULTS Among 192 patients with RV, 11 (5.7%) were diagnosed with IRV. Seven patients with initially presumed IRV were reclassified as secondary after further evaluation. IRV generally affected adult women. Bilateral ocular involvement and retinal phlebitis were common findings. 72% of patients presented with visual loss, which was severe in 27%. Treatments used included systemic glucocorticoids (82%), additional immunosuppressive agents (27%), intravitreal therapy (37%), panretinal photocoagulation (37%) and pars plana vitrectomy (26%). The annual relapse rate was 0.46. Although final visual acuity was considered good in 86%, 45% experienced worsening and only 27% improved. CONCLUSIONS IRV is a rare sight-threatening condition. Despite intensive local and systemic immunosuppressive treatment, visual improvement is observed in only 27% of cases. When IRV is suspected, a differential diagnosis excluding a systemic disease is always warranted. A multidisciplinary approach and a guided clinical, laboratory and imaging evaluation have proven to be useful to distinguish retinal single-organ vasculitis from secondary forms of RV.
Collapse
Affiliation(s)
- Laura Pelegrín
- Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - José Hernández-Rodríguez
- Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.
| | - Gerard Espinosa
- Uveitis Unit, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - Víctor Llorenç
- Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - Maite Sainz-de-la-Maza
- Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - José R Fontenla
- Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - José A Martínez
- Department of Infectious Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - Maria C Cid
- Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| | - Alfredo Adán
- Uveitis Unit, Department of Ophthalmology, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain
| |
Collapse
|
|
30
|
Testicular Vasculitis: A Sonographic and Pathologic Diagnosis. Case Rep Radiol 2017; 2017:8923621. [PMID: 28246567 PMCID: PMC5299182 DOI: 10.1155/2017/8923621] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2016] [Revised: 12/19/2016] [Accepted: 01/10/2017] [Indexed: 11/17/2022] Open
Abstract
Very little has been published about single-organ vasculitis of the testicle in the radiological literature. Consequently, it is a diagnosis that is unfamiliar to most radiologists. This case report describes the sonographic, pathologic, and laboratory findings of testicular vasculitis and reviews the available literature with regard to this subject.
Collapse
|
|
31
|
Jagwani AV, Fathi NQ, Jailani RF, Zakaria AD. Rare and unusual case of polyarteritis nodosa involving the gastrointestinal tract leading to bowel gangrene. BMJ Case Rep 2017; 2017:bcr2016217865. [PMID: 28062430 PMCID: PMC5256521 DOI: 10.1136/bcr-2016-217865] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 12/23/2016] [Indexed: 11/03/2022] Open
Abstract
Polyarteritis nodosa (PAN) is a systemic necrotising vasculitis preferentially targeting medium-sized arteries and not related with glomerulonephritis or small vessel involvement. Clinical manifestations of PAN are multisystem. The gastrointestinal, renal, cardiac, musculoskeletal, skin and central nervous systems may be involved. The aetiology remains unknown, and the ensuing vasculitis may lead to aneurysm formation and thrombosis in any organs of the body with resultant ischaemia. PAN of the intestines is a relatively common manifestation of this disease but rarely causes bowel ischaemia resulting in necrosis. Here we report a case of a young Chinese patient who presented with an acute abdomen requiring surgery and made good recovery post operatively. He remains free of symptoms while on steroid therapy.
Collapse
Affiliation(s)
- Arvind Vashdev Jagwani
- Department of Surgery, Pusat Perubatan Universiti Kebangsaan Malaysia, Cheras, Kuala Lumpur, Malaysia
| | - Nik Qisti Fathi
- Department of Surgery, University Putra Malaysia, Serdang, Selangor, Malaysia
| | - Ruhi Fadzlyana Jailani
- Department of Surgery, Universiti Sains Islam Malaysia, Nilai, Negeri Sembilan, Malaysia
| | | |
Collapse
|
|
32
|
Isolated breast vasculitis manifested as breast edema with suggestive sonographic findings: a case report with imaging findings. J Med Ultrason (2001) 2016; 44:191-195. [PMID: 27830422 DOI: 10.1007/s10396-016-0753-6] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2016] [Accepted: 09/27/2016] [Indexed: 12/31/2022]
Abstract
Early diagnosis of breast vasculitis (BV) is difficult because this condition is rare and occasionally mimics breast cancer clinically or radiologically. It may present as systemic disease or as an isolated lesion in the breast, without systemic evidence. When vasculitis appears in the breast, it also might manifest as a tumor-like lesion, and in previous cases, tissue acquisition was needed for confirmation of the diagnosis because of BV's resemblance to inflammatory breast cancer. We report a case of isolated BV that was suspected of being inflammatory breast cancer clinically, but manifested as bilateral breast edema on mammography. In this case, sonographic findings included not only nonspecific edema findings that might be seen in other cases, but also suggestive findings of hypoechoic circumferential arterial wall thickening with perivascular fat infiltrations that are similar to the halo sign in large arteries but have not been reported in the breast. These are helpful for presumptive diagnosis of BV using ultrasound.
Collapse
|
|
33
|
Abstract
BACKGROUND The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki's syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals. GOAL The aim of this review is to present the various aspects of childhood vasculitis. MATERIALS AND METHODS Reviews and special original papers on childhood vasculitis, published classification criteria and current therapy guidelines were reviewed and summarized. RESULTS The classification of vasculitides in childhood has been modified from the previous adult Chapel Hill classification for vasculitides in 2008. Most therapy recommendations for children are adapted from results of studies in adults. This review covers the current classifications, pathogenesis, clinical manifestations and therapy recommendations for children. DISCUSSION Although etiology and pathogenesis of many vasculitides in childhood are still unknown, clarifying diagnostic methods and effective therapeutic options are available. The knowledge about various forms of disease manifestation may contribute to an early diagnosis and timely initiation of treatment, which may prevent devastating irreversible impairment.
Collapse
|
|
34
|
Gözüküçük M, Gürsoy AY, Kankaya D, Atabekoglu C. Single-organ vasculitis of the cervix accompanying human papillomavirus infection. Interv Med Appl Sci 2016; 8:93-95. [PMID: 28386466 DOI: 10.1556/1646.8.2016.2.111] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/19/2022] Open
Abstract
Single-organ vasculitis (SOV) has rarely been reported to involve the female genital tract but mostly the uterine cervix. A 39-year-old woman was diagnosed to have a high-grade cervical intraepithelial lesion and was treated by large loop excision of the transformation zone. Histopathological evaluation of the excised specimen confirmed the diagnosis of cervical intraepithelial neoplasia grade III accompanied by human papillomavirus infection. The excised second specimen showed the evidence of vasculitis of medium-sized vessels of the cervix, which is a quite rare form of SOV. It seems to be important to be aware of the localized form of polyarteritis nodosa limited to the female genital tract to prevent unnecessary immunosuppressive therapies.
Collapse
Affiliation(s)
- Murat Gözüküçük
- Department of Obstetrics and Gynecology, Kayseri Training and Research Hospital , Kayseri, Turkey
| | - Aslı Yarcı Gürsoy
- Department of Obstetrics and Gynecology, Ufuk University Medical Faculty , Ankara, Turkey
| | - Duygu Kankaya
- Department of Pathology, Ankara University Medical Faculty , Ankara, Turkey
| | - Cem Atabekoglu
- Department of Obstetrics and Gynecology, Ankara University Medical Faculty , Ankara, Turkey
| |
Collapse
|
|
35
|
Fujikawa S, Omoto M, Ogasawara JI, Koga M, Kawai M, Kanda T. [Systemic vasculitic neuropathy diagnosed by means of (18)F-FDG PET CT]. Rinsho Shinkeigaku 2016; 56:88-92. [PMID: 26797481 DOI: 10.5692/clinicalneurol.cn-000803] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/05/2023]
Abstract
We report a 43-year-old man experienced numbness in the distal portion of both legs, which progressed over following two months. Neurological examination showed hypesthesia and muscle weakness in the distal portion of both legs. No abnormal findings were seen on blood test and whole-body contrast enhanced computed tomography (CT). Histopathological findings of the sural nerve and the peroneus brevis muscle showed decreased myelinated nerve fibers with scattered myelin ovoids, vascular occlusion in the epineurium, and inflammatory cell around the arteriole in the muscle bundle. These findings suggested falling in the category as non-systemic vasculitic neuropathy (NSVN). (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed the increase of FDG uptake in the rectum. Inflammatory cell infiltration was found around the arteriole with fibrinoid necrosis in the histopathological specimen of the rectal mucosal biopsy. This result represented the diagnosis as systemic vasculitis. The diagnosis of NSVN may depend on the sensitivity of diagnostic procedure, and (18)F-FDG PET CT might be a useful tool to detect small or medium-sized vasculitis.
Collapse
Affiliation(s)
- Susumu Fujikawa
- Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine
| | | | | | | | | | | |
Collapse
|
|
36
|
Mali P, Muduganti SR, Goldberg J. Rare Case of Vasculitis of the Hepatic Artery. Clin Med Res 2015; 13:169-72. [PMID: 26387709 PMCID: PMC4720514 DOI: 10.3121/cmr.2015.1290] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2015] [Accepted: 06/24/2015] [Indexed: 01/20/2023]
Abstract
Vasculitis is an accumulation of inflammatory leucocytes in the blood vessels with reactive damage to mural structures. Isolated vasculitis of the gastrointestinal tract without systemic involvement is rare. We report a unique case of a female patient who presented with abdominal pain, and was found, on serology, to have elevated inflammatory markers without autoantibodies. A computed tomography scan of the abdomen and pelvis was suggestive of vasculitis of the hepatic artery. To the best of our knowledge, this is the first case, to date, of vasculitis of hepatic artery.
Collapse
Affiliation(s)
- Padmavathi Mali
- Department of Internal Medicine, Marshfield Clinic, Marshfield, Wisconsin USA
| | - Sudheer R Muduganti
- Department of Internal Medicine, Marshfield Clinic, Marshfield, Wisconsin USA
| | - Jerry Goldberg
- Department of Rheumatology, Marshfield Clinic, Marshfield, Wisconsin USA
| |
Collapse
|
|
37
|
Pagnoux C, Khalidi NA. Polyarteritis nodosa – Challenges and options in management. INDIAN JOURNAL OF RHEUMATOLOGY 2015. [DOI: 10.1016/j.injr.2015.08.002] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
|
|
38
|
Affiliation(s)
- Hongmei Xia
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| | - Yan Jiang
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| | - Yuanqing Cai
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| | - Jinliang Tang
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| | - Yunhua Gao
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| | - Jing Ping Sun
- From Department of Ultrasound, Xinqiao Hospital, Third Military Medical University, Chongqing, China (H.X., Y.J., Y.G.); Department of Radiology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (Y.C.); Department of Pathology, Xinqiao Hospital, Third Military Medical University, Chongqing, China (J.T.); Department of Medicine & Therapeutics, Prince of Wales Hospital, Chinese University of Hong Kong (J.S.)
| |
Collapse
|
|
39
|
[Vasculitis: New nomenclature of the Chapel Hill consensus conference 2012]. Z Rheumatol 2015; 73:823-33; quiz 834-5. [PMID: 25269877 DOI: 10.1007/s00393-014-1477-x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022]
Abstract
Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis.
Collapse
|
|
40
|
Yazdani Abyaneh MA, Raghu P, Kircher K, Kutzner H, Alison K, Carlson JA. Circumscribed cicatricial alopecia due to localized sarcoidal granulomas and single-organ granulomatous arteritis: a case report and systematic review of sarcoidal vasculitis. J Cutan Pathol 2015; 42:746-56. [DOI: 10.1111/cup.12530] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2014] [Revised: 10/21/2014] [Accepted: 01/09/2015] [Indexed: 01/14/2023]
Affiliation(s)
| | - Preethi Raghu
- Divisions of Dermatology and Dermatopathology; Albany Medical College; Albany NY USA
| | | | - Heinz Kutzner
- Dermatopathologie; Dermatopathologie Friedrichshafen; Friedrichshafen Germany
| | | | - John Andrew Carlson
- Divisions of Dermatology and Dermatopathology; Albany Medical College; Albany NY USA
| |
Collapse
|
|
41
|
Isolated pulmonary vasculitis: Case report and literature review. Semin Arthritis Rheum 2015; 44:514-517. [DOI: 10.1016/j.semarthrit.2014.10.003] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/06/2014] [Revised: 09/30/2014] [Accepted: 10/10/2014] [Indexed: 11/21/2022]
|
|
42
|
Kanzawa Y, Imai Y, Mizuno Y, Nishioka H. Testicular lymphocytic vasculitis treated with prednisolone and azathioprine. Mod Rheumatol 2015; 27:705-707. [PMID: 25736359 DOI: 10.3109/14397595.2015.1026020] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/13/2022]
Abstract
Testicular vasculitis is a rare condition and little is known about its morphological features. Herein, we report a case of testicular lymphocytic vasculitis, which is rarely documented, in an elderly man. He presented with left testicular swelling and fever, but without any signs of other organ involvement. He was effectively treated with prednisolone and azathioprine. This case report offers information related to the disease course and the importance of biopsy.
Collapse
Affiliation(s)
- Yohei Kanzawa
- a Department of General Internal Medicine , Kobe City Medical Center General Hospital , Kobe, Hyogo , Japan
| | - Yukihiro Imai
- b Department of Pathology , Kobe City Medical Center General Hospital , Kobe, Hyogo , Japan
| | - Yasushi Mizuno
- a Department of General Internal Medicine , Kobe City Medical Center General Hospital , Kobe, Hyogo , Japan
| | - Hiroaki Nishioka
- a Department of General Internal Medicine , Kobe City Medical Center General Hospital , Kobe, Hyogo , Japan
| |
Collapse
|
|
43
|
|
|
44
|
|
|
45
|
Abstract
Inflammatory tumors of the kidney are uncommon and include primary inflammatory processes and systemic diseases such as sarcoidosis, IgG4 disease, and granulomatosis with polyangiitis (GPA). There are approximately 15 cases of the latter in the literature. Tumors in GPA are well described and have been reported in several organs including breast, orbit, mediastinum, central nervous system, and especially the lung. We report the case of a 48-year-old woman who presented with diffuse frontal headaches. Imaging showed both a cranial/sinus and renal mass. The pathology of the dura and nasal sinus biopsies were unrevealing. A nephrectomy was performed that demonstrated a discrete lesion with extensive necrosis, granulomatous inflammation, and crescentic pauci-immune glomerulonephritis, findings consistent with GPA.
Collapse
|
|
46
|
Hernández-Rodríguez J, Tan CD, Rodríguez ER, Hoffman GS. Single-organ gallbladder vasculitis: characterization and distinction from systemic vasculitis involving the gallbladder. An analysis of 61 patients. Medicine (Baltimore) 2014; 93:405-413. [PMID: 25500710 PMCID: PMC4602437 DOI: 10.1097/md.0000000000000205] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Systemic vasculitis (SV) involving abdominal structures usually has a poor prognosis. Gallbladder vasculitis (GV) has been reported as part of SV (GB-SV) and focal single-organ vasculitis (GB-SOV). We analyzed clinical and histologic characteristics of patients with GV to identify features that differentiate GB-SOV from the systemic forms of GV. To identify affected patients with GV we used pathology databases from our institution and an English-language PubMed search. Clinical manifestations, laboratory and histologic features, treatment administered, and outcomes were recorded. Patients were divided in 2 groups, GB-SOV and GB-SV. As in previous studies of single-organ vasculitis, GB-SOV was only considered to be a sustainable diagnosis if disease beyond the gallbladder was not apparent after a follow-up period of at least 6 months. Sixty-one well-characterized patients with GV were included (6 from our institution). There was no significant sex bias (32 female patients, 29 male). Median age was 52 years (range, 18-94 yr). GB-SOV was found in 20 (33%) and GB-SV in 41 (67%) patients. No differences were observed in age, sex frequency, or duration of gallbladder symptoms between groups. Past episodes of recurrent right-upper quadrant or abdominal pain and lithiasic cholecystitis were more frequent in GB-SOV patients, whereas acalculous cholecystitis occurred more often in GB-SV. In GB-SV, gallbladder-related symptoms occurred more often concomitantly with or after the systemic features, but they sometimes appeared before SV was fully developed (13.5%). Constitutional and musculoskeletal symptoms were reported only in GB-SV patients. Compared to GB-SOV, GB-SV patients presented more often with fever (62.5% vs 20%; p = 0.003) and exhibited higher erythrocyte sedimentation rate levels (80 ± 28 vs 37 ± 25 mm/h, respectively; p = 0.006). All GB-SV patients required glucocorticoids and 50% of them also received cytotoxic agents. Mortality in GB-SV was higher than in GB-SOV (35.5% vs 10%; p = 0.05). Nongranulomatous inflammation with fibrinoid necrosis of medium-sized vessels occurred equally in both groups (>90%). Forms of SV affecting the gallbladder included polyarteritis nodosa (n = 10), hepatitis B virus-associated vasculitis (n = 8), cryoglobulinemic (essential or hepatitis C virus-associated) vasculitis (n = 6), vasculitis associated with autoimmune diseases (n = 6), microscopic polyangiitis (n = 4), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (n = 4), IgA vasculitis (Henoch-Schönlein) (n = 2), and giant cell arteritis (n = 1).GV is uncommon. Its histology most often consists of a nongranulomatous necrotizing vasculitis affecting medium-sized vessels. GB-SOV is usually discovered after routine cholecystectomy performed because of the presence of local symptoms, gallstone-associated cholecystitis, and contrary to GB-SV, GB-SOV is usually not associated with systemic symptoms. Acute phase reactants and surrogate markers of autoimmunity are usually normal or negative in GB-SOV. GB-SOV does not require systemic antiinflammatory or immunosuppressive therapy; surgery is adequate to achieve cure. GB-SV always warrants immunosuppressant therapy and is associated with high mortality. The finding of GV may precede the generalized manifestations of SV. Therefore, once GV is discovered, studies to determine disease extent and a vigilant follow-up are mandatory.
Collapse
Affiliation(s)
- José Hernández-Rodríguez
- From the Vasculitis Research Unit, Department of Autoimmune Diseases, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain (JHR); Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases (GSH); and Department of Anatomic Pathology (CDT, ERR), Cleveland Clinic, Cleveland, Ohio, United States
| | | | | | | |
Collapse
|
|
47
|
Roma AA, Amador-Ortiz C, Liapis H. Significance of isolated vasculitis in the gynecological tract: what clinicians do with the pathologic diagnosis of vasculitis? Ann Diagn Pathol 2014; 18:199-202. [DOI: 10.1016/j.anndiagpath.2014.03.008] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2014] [Accepted: 03/13/2014] [Indexed: 11/26/2022]
|
|
48
|
[Vasculitis. New nomenclature of the Chapel Hill consensus conference 2012]. DER PATHOLOGE 2014; 34:569-79; quiz 580-1. [PMID: 24154756 DOI: 10.1007/s00292-013-1796-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/27/2022]
Abstract
In recent years, many advances have been made in our understanding of vasculitis etiopathology as well as of different disease courses. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature reflects current knowledge about etiopathology, in addition to the descriptive principles of vessel size and type of inflammation. Anti-neutrophil cyptoplasmic antibody (ANCA)-associated vasculitides have been classified as a separate group, as opposed to immune complex small vessel vasculitis. In cases where consensus was achieved, eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes, focussing on those types that are relevant to the histopathologist, and summarizes the results of important new articles on morphology and clinical picture of vasculitis.
Collapse
|
|
49
|
Abstract
The vasculitides are a large group of heterogeneous diseases for which it has been assumed that pathogenesis is largely autoimmune. As clinicians, we distinguish one form of vasculitis from another on the basis of observed patterns of organ injury, the size of the vessels affected and histopathological findings. The terms 'small-vessel', 'medium-vessel' and 'large-vessel' vasculitis are useful clinical descriptors, but fail to inform us about why vessels of a certain calibre are favoured by one disease and not another. Classification based on vessel size also fails to consider that vessels of a specific calibre are not equally prone to injury. Distinct vulnerabilities undoubtedly relate to the fact that same-size vessels in different tissues may not be identical conduits. In fact, vessels become specialized, from the earliest stages of embryonic development, to suit the needs of different anatomical locations. Vessels of the same calibre in different locations and organs are as different as the organ parenchymal cells through which they travel. The dialogue between developing vessels and the tissues they perfuse is designed to meet special local needs. Added to the story of vascular diversity and vulnerability are changes that occur during growth, development and ageing. An improved understanding of the unique territorial vulnerabilities of vessels could form the basis of new hypotheses for the aetiopathogenesis of the vasculitides. This Review considers how certain antigens, including infectious agents, might become disease-relevant and how vascular diversity could influence disease phenotypes and the spectrum of vascular inflammatory diseases.
Collapse
Affiliation(s)
- Gary S Hoffman
- Department of Rheumatic and Immunologic Diseases, A50, 9500 Euclid Avenue, Lerner College of Medicine, Cleveland Clinic, Cleveland, OH 44195, USA
| | - Leonard H Calabrese
- Department of Rheumatic and Immunologic Diseases, A50, 9500 Euclid Avenue, Lerner College of Medicine, Cleveland Clinic, Cleveland, OH 44195, USA
| |
Collapse
|
|
50
|
Lintern N, Johnson NR, Mckenzie I, Martin B. Testicular vasculitis - literature review and case report in queensland. Curr Urol 2014; 7:107-9. [PMID: 24917768 DOI: 10.1159/000356258] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/24/2013] [Accepted: 02/15/2013] [Indexed: 01/16/2023] Open
Abstract
A 21-year-old male presented with acute onset, sharp right sided testicular pain. The testicle was removed with a histological diagnosis of testicular vasculitis. Anti-neutrophil cytoplasmic antibodies were negative. Although rare, males who present with acute onset pain should be screened for testicular vasculitis with a scrotal ultrasound and blood investigations including tumor markers and anti-neutrophil cytoplasmic antibodies.
Collapse
Affiliation(s)
- Narelle Lintern
- Urology Department, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
| | - Nigel R Johnson
- Urology Department, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
| | - Ian Mckenzie
- Urology Department, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
| | - Ben Martin
- Urology Department, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia
| |
Collapse
|