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Lopez-Gomez M, Moya-Alvarado P, Park HS, Martín MC, Calleja S, Codes-Mendez H, Magallares B, Castellví I, Barros-Membrilla AJ, Laiz A, Diaz-Torné C, Sainz L, Bernárdez J, Martínez-Martinez L, Corominas H. Comparative Analysis of Classification Criteria in IgG4-Related Disease and Evaluating Diagnostic Accuracy from a Retrospective Cohort in Clinical Practice. Diagnostics (Basel) 2024; 14:2583. [PMID: 39594249 PMCID: PMC11593256 DOI: 10.3390/diagnostics14222583] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/17/2024] [Revised: 11/12/2024] [Accepted: 11/14/2024] [Indexed: 11/28/2024] Open
Abstract
INTRODUCTION We conducted a comprehensive comparative analysis of the Okazaki, Umehara, and American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria for diagnosing immunoglobulin G4-related disease (IgG4-RD). MATERIALS AND METHODS A retrospective study was conducted in a single tertiary hospital, using expert clinical judgment as the gold standard. We compared the diagnostic accuracy of the Okazaki, Umehara, and ACR/EULAR criteria in a cohort of 41 patients with suspected IgG4-RD. We assessed sensitivity, specificity, and positive and negative predictive values for each criterion, and conducted a separate analysis based on four IgG4-RD subtypes. RESULTS A total of 30 patients were confirmed to have IgG4-RD and 11 were identified as mimickers. The Umehara criteria demonstrated the highest sensitivity (83.33%), followed by the ACR/EULAR 2019 (66.67%) and Okazaki (60.0%) criteria. All three criteria exhibited 100% specificity, with overall diagnostic accuracy ranging from 70% to 88%. The areas under the curve (AUC) were 0.917 (Umehara), 0.800 (Okazaki), and 0.833 (ACR/EULAR 2019), indicating significant diagnostic effectiveness (p < 0.000). Subtype analysis revealed that the Umehara and ACR/EULAR 2019 criteria were more effective in diagnosing pancreato-hepato-biliary involvement (subtype 1), while the Okazaki and ACR/EULAR 2019 criteria were more effective in diagnosing retroperitoneal fibrosis and/or aortitis (subtype 2). CONCLUSIONS Our study provides valuable insights into the diagnostic performance of the Okazaki, Umehara, and ACR/EULAR criteria for a cohort of patients with suspected IgG4-RD. The Umehara criterion demonstrated the highest sensitivity, suggesting its potential utility for screening purposes, while all three criteria showed consistent specificity.
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Affiliation(s)
- Marta Lopez-Gomez
- Rheumatology Department, Hospital Universitario Araba, 01009 Vitoria, Spain
- Instituto de Investigación Biomédica BIORABA, Hospital Universitario Araba, 01009 Vitoria, Spain
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
| | - Patricia Moya-Alvarado
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Hye Sang Park
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Mar Concepción Martín
- Gastroenterology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Sara Calleja
- Immunology Department, Hospital de la Santa Creu i Sant Pau, IIB Sant Pau, UAB, 08025 Barcelona, Spain; (S.C.); (L.M.-M.)
| | - Helena Codes-Mendez
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Berta Magallares
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Iván Castellví
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | | | - Ana Laiz
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - César Diaz-Torné
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Luis Sainz
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Julia Bernárdez
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
| | - Laura Martínez-Martinez
- Immunology Department, Hospital de la Santa Creu i Sant Pau, IIB Sant Pau, UAB, 08025 Barcelona, Spain; (S.C.); (L.M.-M.)
| | - Hèctor Corominas
- Department of Medicine, Universitat Autònoma de Barcelona (UAB), 08193 Barcelona, Spain; (H.C.-M.); (B.M.); (I.C.); (H.C.)
- Institut de Recerca Sant Pau (IR SANT PAU), 08041 Barcelona, Spain; (H.S.P.); (A.L.); (C.D.-T.); (L.S.)
- Rheumatology Department, Hospital de la Santa Creu i Sant Pau, 08025 Barcelona, Spain;
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Martínez Calabuig P, Fragío Gil JJ, González Mazarío R, López Gutiérrez F, Loricera García J, Blanco Alonso R, Campos Fernández C. IgG4-related disease: Effectiveness evaluation through Umehara-Okazaki 2011 and ACR/EULAR 2019 diagnostic criteria. Med Clin (Barc) 2024; 163:217-223. [PMID: 38849269 DOI: 10.1016/j.medcli.2024.03.026] [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: 12/11/2023] [Revised: 03/25/2024] [Accepted: 03/26/2024] [Indexed: 06/09/2024]
Abstract
BACKGROUND IgG4-related disease (IgG4-RD) is a rare, systemic immune-mediated fibro-inflammatory condition with an unclear etiology and pathophysiology, potentially affecting multiple organs. It presents with common clinical, radiological, and serological characteristics. This study aims to compare the latest two IgG4-RD classification and diagnostic criteria: Umehara-Okazaki 2011 and ACR/EULAR 2019. MATERIAL AND METHODS In a retrospective cross-sectional study conducted across two centers from January 2010 to July 2023, we included patients suspected of having IgG4-RD from various hospital departments. Patients finally diagnosed with other pathologies were excluded. The remaining suspected IgG4-RD cases were evaluated using both Umehara-Okazaki 2011 and ACR/EULAR 2019 criteria. RESULTS Out of 34 patients with a clinical diagnosis of IgG4-RD, the Umehara-Okazaki 2011 classified 20 patients: 5 as definitive, 7 as probable, and 8 as possible cases. Applying the ACR/EULAR 2019 criteria to the same cohort resulted in the diagnosis of 9 patients. Notably, retroperitoneal fibrosis and aortitis were the most prevalent form of presentation, accounting for 25% and 22.2% of cases classified under the 2011 and 2019 criteria, respectively. DISCUSSION The more recent and stringent ACR/EULAR 2019 criteria focus on histopathology, various forms of presentation, and analytical data, allow for a more accurate classification of patients.
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Affiliation(s)
- Pablo Martínez Calabuig
- Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain.
| | - Jorge Juan Fragío Gil
- Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain
| | - Roxana González Mazarío
- Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain
| | | | - Javier Loricera García
- Servicio de Reumatología, Hospital Marqués de Valdecilla, Santander, Spain; IDIVAL, Spain
| | - Ricardo Blanco Alonso
- Servicio de Reumatología, Hospital Marqués de Valdecilla, Santander, Spain; IDIVAL, Spain
| | - Cristina Campos Fernández
- Servicio de Reumatología y Metabolismo Óseo, Consorcio Hospital General Universitario, Valencia, Spain; Escuela de Doctorado, Universidad Católica de Valencia San Vicente Mártir (UCV), Valencia, Spain
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Bateman AC, Culver EL. Challenges and pitfalls in the diagnosis of IgG4-related disease. Semin Diagn Pathol 2024; 41:45-53. [PMID: 38000975 DOI: 10.1053/j.semdp.2023.11.005] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/30/2023] [Accepted: 11/14/2023] [Indexed: 11/26/2023]
Abstract
IgG4-related disease (IgG4-RD) is a relatively novel fibroinflammatory condition characterized typically by dense lymphoplasmacytic inflammation, storiform fibrosis and obliterative venulitis, together with prominent IgG4+ plasma cells and an IgG4+/IgG+ plasma cell ratio of >40 %. The diagnosis is usually made on a combination of clinical and serological features together with characteristic radiological and histological appearances. The condition may be limited to a single tissue/organ (e.g., autoimmune pancreatitis) or may be multicentric in nature - four clinical 'patterns' of disease distribution have recently been described. The diagnosis of IgG4-RD can be challenging, particularly when the clinical presentation is unusual and/or when the histological features are not typical. A diagnosis of IgG4-RD may still be achieved in these situations, after careful clinicopathological discussion e.g., at a specialist multidisciplinary team meeting. However, a wide range of other conditions (neoplastic and non-neoplastic) can mimic IgG4-RD, clinically and/or on histological examination. The relationship between IgG4-RD and non-IgG4-RD associated conditions in some clinical situations is particularly complex. This review describes the role of histological examination in the diagnosis of IgG4-RD, discusses some of the practical difficulties that may be encountered and provides an insight into the range of non-IgG4-RD associated conditions that can mimic IgG4-RD on clinical and/or histological grounds. The requirement for interpretation of histological features in the context of the global clinical picture of the patient is highlighted and emphasized.
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Affiliation(s)
- Adrian C Bateman
- Department of Cellular Pathology, Southampton General Hospital, MP002, Level E, South Block, Tremona Road, Southampton SO16 6YD, UK.
| | - Emma L Culver
- Translational Gastroenterology Unit, John Radcliffe Hospital, Oxford, UK
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Mukoyama H, Murakami K, Onizawa H, Shirakashi M, Hiwa R, Tsuji H, Kitagori K, Akizuki S, Nakashima R, Onishi A, Yoshifuji H, Tanaka M, Morinobu A. A case of atypical IgG4-related disease presenting hypereosinophilia, polyneuropathy, and liver dysfunction. Mod Rheumatol Case Rep 2023; 8:172-177. [PMID: 37750557 DOI: 10.1093/mrcr/rxad053] [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: 04/21/2023] [Revised: 06/18/2023] [Accepted: 09/03/2023] [Indexed: 09/27/2023]
Abstract
Ig (immunoglobulin) G4-related disease (Ig4-RD) affects several organs, including salivary glands, lacrimal glands, pancreas, biliary ducts, and retroperitoneum. A 72-year-old woman was examined for hypereosinophilia, high levels of IgG4, polyneuropathy, liver dysfunction, enlargement of lymph nodes and lacrimal glands, and beaded dilation of the bile ducts. We diagnosed Ig4-RD based on biopsies of the lymph nodes, liver, and submandibular gland. The symptoms of the patient improved after glucocorticoid treatment. This was a novel and atypical case of Ig4-RD that was difficult to differentiate from other diseases, including eosinophilic granulomatosis with polyangiitis, idiopathic hypereosinophilic syndrome, and polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes syndrome. This case report highlights the importance of biopsies in differentiating Ig4-RD.
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Affiliation(s)
- Hiroki Mukoyama
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Kosaku Murakami
- Center for Cancer Immunotherapy and Immunobiology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hideo Onizawa
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Mirei Shirakashi
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Ryosuke Hiwa
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hideaki Tsuji
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Koji Kitagori
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Shuji Akizuki
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Ran Nakashima
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akira Onishi
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Hajime Yoshifuji
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Masao Tanaka
- Department of Advanced Medicine for Rheumatic Diseases, Graduate School of Medicine, Kyoto University, Kyoto, Japan
| | - Akio Morinobu
- Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan
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Czerniak S, Rao A, Mathur M. Multimodality pictorial review of IgG4-related disease in the abdomen and pelvis. Abdom Radiol (NY) 2023; 48:3147-3161. [PMID: 37407744 DOI: 10.1007/s00261-023-03996-w] [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: 04/13/2023] [Revised: 06/20/2023] [Accepted: 06/21/2023] [Indexed: 07/07/2023]
Abstract
BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated disease that can affect multiple organs, including the orbits, salivary glands, thyroid gland, lungs, aorta, pancreas, bile ducts, lymph nodes, and retroperitoneum. While timely diagnosis is particularly important given the efficacy of glucocorticoid treatment for IgG4-RD, accurate recognition can prove a challenge given the overlap between the imaging features of this disease and other entities. PURPOSE After a review of the epidemiology, pathophysiology, and clinical considerations (including treatment) associated with IgG4-RD, this pictorial review will showcase the variable imaging manifestations of this disease in the abdomen and pelvis. Post-treatment imaging appearance of these entities will be reviewed and mimickers of this disease in the abdomen and pelvis will be presented. CONCLUSION The presence of mass-like soft tissue with radiographic characteristics of fibrosis affecting multiple organs should raise suspicion for IgG4-RD, although definite diagnosis can only be made with appropriate clinical, serological, and pathologic data.
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Affiliation(s)
- Suzanne Czerniak
- Department of Radiology and Biomedical Imaging, Yale School of Medicine, 333 Cedar Street, Room TE-2, PO Box 208042, New Haven, CT, 06520, USA.
| | - Aditya Rao
- Diagnostic Radiology Resident, Yale-New Haven Hospital, New Haven, CT, USA
| | - Mahan Mathur
- Department of Radiology and Biomedical Imaging, Yale School of Medicine, 333 Cedar Street, Room TE-2, PO Box 208042, New Haven, CT, 06520, USA
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Ravanbakhsh N, Shillingford N, Piester TL. A Diagnostic Conundrum: A Case of Pediatric Autoimmune Pancreatitis. JPGN REPORTS 2023; 4:e326. [PMID: 37600616 PMCID: PMC10435041 DOI: 10.1097/pg9.0000000000000326] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 12/14/2022] [Accepted: 04/14/2023] [Indexed: 08/22/2023]
Abstract
Autoimmune pancreatitis (AIP) is rare cause of abdominal pain in children who often present with obstructive jaundice, mimicking malignancy. An investigation of clinical symptoms, serology, imaging, and histopathology is necessary for diagnosis. We report a 10-year-old female presenting with abdominal pain and jaundice, ultimately found to have AIP after confirmation with tissue pathology. Our patient's prompt response to corticosteroid initiation is characteristic of this disease state. AIP has 2 subtypes, the second of which is more frequently found in children. Our patient's pathology did not fit perfectly with either subtype, but had features found in each one. While diagnostic criteria for AIP have not established in pediatrics, our case highlights the combination of clinical symptoms, imaging, and histopathology that children classically present with. While rare, the diagnosis of AIP is associated with comorbidities and must be considered in any child presenting with a pancreatic mass or biliary stricture.
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Affiliation(s)
- Naseem Ravanbakhsh
- From the Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Los Angeles, Los Angeles, CA, USA
| | - Nick Shillingford
- Division of Pathology, Children’s Hospital Los Angeles, Los Angeles, CA, USA
| | - Travis L. Piester
- From the Division of Gastroenterology, Hepatology and Nutrition, Children’s Hospital Los Angeles, Los Angeles, CA, USA
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Uversky VN, Redwan EM, Makis W, Rubio-Casillas A. IgG4 Antibodies Induced by Repeated Vaccination May Generate Immune Tolerance to the SARS-CoV-2 Spike Protein. Vaccines (Basel) 2023; 11:vaccines11050991. [PMID: 37243095 DOI: 10.3390/vaccines11050991] [Citation(s) in RCA: 38] [Impact Index Per Article: 19.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2023] [Revised: 05/12/2023] [Accepted: 05/15/2023] [Indexed: 05/28/2023] Open
Abstract
Less than a year after the global emergence of the coronavirus SARS-CoV-2, a novel vaccine platform based on mRNA technology was introduced to the market. Globally, around 13.38 billion COVID-19 vaccine doses of diverse platforms have been administered. To date, 72.3% of the total population has been injected at least once with a COVID-19 vaccine. As the immunity provided by these vaccines rapidly wanes, their ability to prevent hospitalization and severe disease in individuals with comorbidities has recently been questioned, and increasing evidence has shown that, as with many other vaccines, they do not produce sterilizing immunity, allowing people to suffer frequent re-infections. Additionally, recent investigations have found abnormally high levels of IgG4 in people who were administered two or more injections of the mRNA vaccines. HIV, Malaria, and Pertussis vaccines have also been reported to induce higher-than-normal IgG4 synthesis. Overall, there are three critical factors determining the class switch to IgG4 antibodies: excessive antigen concentration, repeated vaccination, and the type of vaccine used. It has been suggested that an increase in IgG4 levels could have a protecting role by preventing immune over-activation, similar to that occurring during successful allergen-specific immunotherapy by inhibiting IgE-induced effects. However, emerging evidence suggests that the reported increase in IgG4 levels detected after repeated vaccination with the mRNA vaccines may not be a protective mechanism; rather, it constitutes an immune tolerance mechanism to the spike protein that could promote unopposed SARS-CoV2 infection and replication by suppressing natural antiviral responses. Increased IgG4 synthesis due to repeated mRNA vaccination with high antigen concentrations may also cause autoimmune diseases, and promote cancer growth and autoimmune myocarditis in susceptible individuals.
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Affiliation(s)
- Vladimir N Uversky
- Department of Molecular Medicine and USF Health Byrd Alzheimer's Research Institute, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA
| | - Elrashdy M Redwan
- Biological Science Department, Faculty of Science, King Abdulaziz University, P.O. Box 80203, Jeddah 21589, Saudi Arabia
- Therapeutic and Protective Proteins Laboratory, Protein Research Department, Genetic Engineering and Biotechnology Research Institute, City for Scientific Research and Technology Applications, New Borg EL-Arab, Alexandria 21934, Egypt
| | - William Makis
- Cross Cancer Institute, Alberta Health Services, 11560 University Avenue, Edmonton, AB T6G 1Z2, Canada
| | - Alberto Rubio-Casillas
- Autlan Regional Hospital, Health Secretariat, Autlan 48900, Jalisco, Mexico
- Biology Laboratory, Autlan Regional Preparatory School, University of Guadalajara, Autlan 48900, Jalisco, Mexico
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Wu H, Lei D, Zhang X, Wang M, Wang Y, Xia J, Chen F, Chen B, Tian Y. Effects of Fibulin-5 Gene Silencing on Proliferation and Apoptosis of IgG4-ROD Lacrimal Gland Fibroblasts. Stem Cells Int 2023; 2023:2742839. [PMID: 36818161 PMCID: PMC9937754 DOI: 10.1155/2023/2742839] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2022] [Revised: 09/13/2022] [Accepted: 10/12/2022] [Indexed: 02/12/2023] Open
Abstract
Objective This study is aimed at discussing the value of RNA interference technology on inhibiting lacrimal gland fibrosis in IgG4-related ocular disease (IgG4-ROD). Methods Six patients with IgG4-ROD who came to the hospital for surgical treatment from October 2018 to August 2019 were selected, and their diseased lacrimal glands were taken for primary cell culture and fibroblast identification. High efficiency and specificity small interference RNA (siRNA) plasmid vector was constructed, its inhibitory effect on fibroblast proliferation was determined by CCK-8 assay, and the appropriate concentration was selected as the siRNA concentration for subsequent experiments. RT-PCR and Western blot detected the relative expression levels of Fibulin-5 mRNA and protein in the cells 48 hours after transfection. The apoptosis rate of each group of cells at 24 hours, 48 hours, and 72 hours after transfection was detected by flow cytometry, and the proliferation and apoptosis of cells after silencing Fibulin-5 were analyzed and compared. Results 24 hours after transfection, there was no significant difference in the proliferation rate among the four groups (P > 0.05); 48 hours and 72 hours after Fibulin-5 siRNA transfection, the proliferation activity of the transfected cells was significantly decreased compared with the 0 nM group, and the inhibitory effect of 75 nM siRNA was the strongest. The expression of Fibulin-5 mRNA and protein in the siRNA-transfected cells was significantly decreased compared with the blank and empty vector negative siRNA groups, and the difference was statistically significant (P < 0.05). The apoptosis rate of cells in the Fibulin-5 siRNA transfection group was significantly higher than that of cells in the blank and empty vector negative siRNA groups, and the difference was statistically significant (P < 0.05). Conclusion Fibulin-5 siRNA recombinant plasmid can significantly downregulate the mRNA and protein expressions of target gene Fibulin-5 and promote apoptosis after transfection into IgG4-ROD lacrimal gland fibroblasts. It is speculated that Fibulin-5 can be used as a target to effectively inhibit the fibrosis of lacrimal gland tissues by RNAi technique.
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Affiliation(s)
- Huarong Wu
- Department of Ophthalmology, Anqing Municipal Hospital, Anqing, China
| | - Daikun Lei
- Department of Ophthalmology, Beijing Road Medical District, General Hospital of Xinjiang Military Region, Urumqi, Xinjiang, China
| | - Xiaoling Zhang
- Department of Ophthalmology, Beijing Road Medical District, General Hospital of Xinjiang Military Region, Urumqi, Xinjiang, China
| | - Mengfei Wang
- AIER Eye Hospital Group, Sichuan Eye Hospital, Sichuan, China
| | - Yuanyuan Wang
- Department of Ophthalmology, The First People's Hospital of Pinghu (Pinghu Hospital Affiliated to Hangzhou Medical University), Pinghu, China
| | - Jie Xia
- Department of Ophthalmology, Lujiang County People's Hospital, Hefei, China
| | - Fan Chen
- Department of Ophthalmology, Anqing Municipal Hospital, Anqing, China
| | - Bei Chen
- Department of Ophthalmology, Anqing Municipal Hospital, Anqing, China
| | - Yanming Tian
- Department of Ophthalmology, Beijing Road Medical District, General Hospital of Xinjiang Military Region, Urumqi, Xinjiang, China
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Jatteppanvar B, Chakravarthy P, K MT, Sudan A, . R. Immunoglobulin G4-Related Disease: A Case Report. Cureus 2022; 14:e31403. [DOI: 10.7759/cureus.31403] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/11/2022] [Indexed: 11/13/2022] Open
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Miyagi A, Fujimoto D, Yoshikawa A, Okushi S, Miyagi J, Matsumoto R, Ogata S, Bando Y, Ogawa H, Tanahashi T. A rare case of fibrohistiocytic hepatic inflammatory pseudotumor with cholecystocholangitis showing positive IgG4 staining. Clin J Gastroenterol 2022; 15:975-980. [DOI: 10.1007/s12328-022-01663-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2022] [Accepted: 06/12/2022] [Indexed: 11/29/2022]
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Ganta N, Prasad A, Gupta V, Kochhar S, Pavuluri S, Ghodasara K, Cheriyath P. A Case Report of a Prevertebral Mass in an Elderly Male Post Hodgkin's Lymphoma. Cureus 2022; 14:e28494. [PMID: 36185854 PMCID: PMC9514051 DOI: 10.7759/cureus.28494] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/27/2022] [Indexed: 11/13/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory autoimmune disease characterized by tissue infiltration by dense lymphoplasmacytic infiltrate composed of T cells, activated B-cells, and plasma cells expressing IgG4 and has varied presentations with similar histopathology. It can involve visceral organs, glands, aorta, lymph nodes, and retroperitoneal tissue. In our case, a 68-year-old male with a past history of Hodgkin's lymphoma and in remission presented for investigation of polyclonal gammopathy. Serum electrophoresis showed increased free kappa light chains, free lambda light chains, and kappa lambda ratio; immunoglobulin G (IgG) levels were also increased. A positron emission tomography (PET) scan and magnetic resonance imaging (MRI) thoracic spine suggested a hypermetabolic prevertebral soft tissue density. Biopsy of the mass suggested IgG4-related disease (IgG4-RD). He also had a compression fracture of the T7 vertebra. He was started on intravenous methylprednisolone and rituximab, following which he had a significant decrease in the size of the mass along with a decline in the levels of IgG, kappa, and lambda chains.
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Complemental Diagnosis of IgG4-Related Pancreaticobiliary Diseases by Multiple Hypoechoic Lesions in the Submandibular Glands. J Clin Med 2022; 11:jcm11144189. [PMID: 35887953 PMCID: PMC9319748 DOI: 10.3390/jcm11144189] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2022] [Accepted: 07/18/2022] [Indexed: 12/04/2022] Open
Abstract
The diagnosis of autoimmune pancreatitis (AIP) and immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) may require a somewhat invasive pathological examination and steroid responsiveness. This retrospective study assessed the complemental diagnosis of AIP and IgG4-SC using submandibular gland (SG) ultrasonography (US) in 69 patients, including 54 patients with AIP, 2 patients with IgG4-SC, and 13 patients with both AIP and IgG4-SC. The data from the physical examination and US of SGs to diagnose AIP (n = 67) and IgG4-SC (n = 15) were analyzed. The steroid therapy efficacy in resolving hypoechoic lesions in SGs was evaluated in 36 cases. The presence of IgG4-related pancreaticobiliary disease with multiple hypoechoic lesions in SGs was reduced from 31 to 11 cases after steroid therapy, suggesting that multiple hypoechoic lesions in SGs are strongly associated with IgG4-positive cell infiltrations. Multiple hypoechoic lesions in SGs were observed in 53 cases, whereas submandibular swelling on palpation was observed in 21 cases of IgG4-related pancreaticobiliary diseases. A complemental diagnosis of IgG4-related pancreaticobiliary diseases without a histological diagnosis and steroid therapy was achieved in 57 and 68 cases without and with multiple hypoechoic lesions in SGs, respectively. In conclusion, multiple hypoechoic lesions in SGs are useful for the complemental diagnosis of IgG4-related pancreaticobiliary diseases.
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13
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Orchestration of Immune Cells Contributes to Fibrosis in IgG4-Related Disease. IMMUNO 2022. [DOI: 10.3390/immuno2010013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
This review summarizes recent progress in understanding the pathogenesis of IgG4-related disease (IgG4-RD), with a focus on fibrosis. Several studies reported that CD4+ T cells with cytotoxic activity promoted by the secretion of granzyme and perforin, cytotoxic CD4+ T cells (CD4+CTLs), and disease-specific activated B cells, infiltrated inflamed tissues and cooperated to induce tissue fibrosis in autoimmune fibrotic diseases such as IgG4-RD, systemic sclerosis, and fibrosing mediastinitis. An accumulation of cells undergoing apoptotic cell death induced by CD4+CTLs and CD8+CTLs followed by macrophage-mediated clearing and finally tissue remodeling driven by cytokines released by CD4+CTLs, activated B cells, and M2 macrophages may contribute to the activation of fibroblasts and collagen production. In IgG4-RD, this process likely involves the apoptosis of non-immune, non-endothelial cells of mesenchymal origin and subsequent tissue remodeling. In summary, CD4+CTLs infiltrate affected tissues where they may cooperate with activated B cells, CD8+CTLs, and M2 macrophages, to induce apoptosis by secreting cytotoxic cytokines. These immune cells also drive fibrosis by secreting pro-fibrotic molecules in IgG4-RD.
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14
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IgG4-related pseudotumours: a series of 12 cases and a review of the literature. Pathology 2022; 54:563-572. [DOI: 10.1016/j.pathol.2021.11.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/01/2021] [Revised: 11/11/2021] [Accepted: 11/18/2021] [Indexed: 11/22/2022]
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15
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Yu T, Wu Y, Liu J, Zhuang Y, Jin X, Wang L. The risk of malignancy in patients with IgG4-related disease: a systematic review and meta-analysis. Arthritis Res Ther 2022; 24:14. [PMID: 34986892 PMCID: PMC8728936 DOI: 10.1186/s13075-021-02652-2] [Citation(s) in RCA: 30] [Impact Index Per Article: 10.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2021] [Accepted: 10/12/2021] [Indexed: 12/23/2022] Open
Abstract
BACKGROUND The relationship between IgG4-related disease (IgG4-RD) and the risk of malignancy is still controversial. This article focused on assessing the risk of cancer in patients with IgG4-RD by meta-analysis. METHODS We conducted a systematic review of the literature and meta-analysis characterizing the associated risk of overall malignancy and four site-specific malignancies (pancreas, lung, gastric and lymphoma) in patients with IgG4-RD. A search from 2003 to 2020 was performed using specified terms from PubMed, Embase, Web of Science and SinoMed. Random-effects model analysis was used to pool standardized incidence ratios (SIRs) and 95% confidence intervals (CIs). Subgroup and sensitivity analyses were conducted to clarify the heterogeneity of the included studies. Begg's funnel plot and Egger's linear regression test were used to evaluate the bias of the meta-analysis. A P value < 0.05 indicated the existence of publication bias. RESULTS A total of 10 studies were included in the article. The overall SIR estimates suggested an increased risk of overall cancer in IgG4-RD patients (SIR 2.57 95% CI 1.72-3.84) compared with the general population. The specific SIRs for pancreas and lymphoma were higher than those of the general population in IgG4-RD patients (SIR 4.07 95% CI 1.04-15.92, SIR 69.17 95% CI 3.91-1223.04, respectively). No significant associations were revealed in respiratory and gastric cancer (SIR 2.14 95% CI 0.97-4.75, SIR 0.95 95% CI 0.24-3.95, respectively). Four studies were found to be the major sources of heterogeneity by sensitivity analysis. There was no evidence of publication bias via Egger's test. CONCLUSION Compared with the general population, patients with IgG4-RD appear to have a higher risk of overall cancer, especially pancreatic and lymphoma. The risk of lung and gastric cancer was not different between IgG4-RD patients and the general population.
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Affiliation(s)
- Tingfeng Yu
- Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Guangzhou, China
| | - Yaxian Wu
- Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Guangzhou, China
| | - Jia Liu
- Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Guangzhou, China
| | - Yanyan Zhuang
- Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Guangzhou, China
| | - Xiaoyan Jin
- Department of General Practice, Sun Yat-sen Memorial Hospital, Guangzhou, China.
| | - Lingyun Wang
- Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Guangzhou, China.
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16
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Suisa H, Soustiel JF, Grober Y. IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case. JOURNAL OF NEUROSURGERY: CASE LESSONS 2021; 2:CASE21398. [PMID: 36061082 PMCID: PMC9435580 DOI: 10.3171/case21398] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 07/07/2021] [Accepted: 07/12/2021] [Indexed: 11/25/2022]
Abstract
BACKGROUND Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition with potential multiorgan involvement. Common manifestations include autoimmune pancreatitis and retroperitoneal fibrosis. Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease. OBSERVATIONS A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month. LESSONS IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.
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Affiliation(s)
- Haggai Suisa
- Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and
| | - Jean Francois Soustiel
- Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and
- The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel
| | - Yuval Grober
- Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and
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17
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Savić Vuković A, Kukuljan M, Dinter M, Jurinović K, Jonjić N. The diagnostic challenge of adenocarcinoma in pulmonary nodular lymphoid hyperplasia. SAGE Open Med Case Rep 2021; 9:2050313X211039371. [PMID: 34721874 PMCID: PMC8552398 DOI: 10.1177/2050313x211039371] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/19/2021] [Accepted: 07/27/2021] [Indexed: 11/16/2022] Open
Abstract
Pulmonary nodular lymphoid hyperplasia is a rare, nonneoplastic lymphoproliferative disorder mostly manifesting as one or more nodules or localized lung infiltrates. The lesion comprises reactive germinal centers with well-preserved mantle zones and sheets of interfollicular mature plasma cells, lymphocytes, histiocytes, and neutrophils. The radiological finding is not specific, and the diagnosis of pulmonary nodular lymphoid hyperplasia relies generally on pathohistological and immunohistochemical analyses. The most important differential diagnoses are extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue and immunoglobulin G4–related sclerosing disease. Nonetheless, we present a case of pulmonary nodular lymphoid hyperplasia in a 69-year-old woman with the diagnostic challenge of cytological atypia in alveolar spaces inside the lymphoid tissue, coexisting with the diagnosis of adenocarcinoma of the lepidic pattern. Therefore, this case highlights the importance of identifying these rare benign and reactive lymphoproliferative diseases given the risk of developing not only lymphoma but also carcinoma.
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Affiliation(s)
| | - Melita Kukuljan
- Clinical Hospital Center Rijeka, KBC Rijeka, Rijeka, Croatia
| | - Morana Dinter
- Department of Cytology, Clinical Hospital Center Rijeka, KBC Rijeka, Rijeka, Croatia
| | | | - Nives Jonjić
- Clinical Hospital Center Rijeka, KBC Rijeka, Rijeka, Croatia.,Department of Pathology, Faculty of Medicine, University of Rijeka, Rijeka, Croatia
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18
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Bastati N, Kristic A, Poetter-Lang S, Messner A, Herold A, Hodge JC, Schindl M, Ba-Ssalamah A. Imaging of inflammatory disease of the pancreas. Br J Radiol 2021; 94:20201214. [PMID: 34111970 PMCID: PMC8248196 DOI: 10.1259/bjr.20201214] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
Increasingly acute and chronic pancreatitis (AP and CP) are considered a continuum of a single entity. Nonetheless, if, after flare-up, the pancreas shows no residual inflammation, it is classified as AP. CP is characterised by a long cycle of worsening and waning glandular inflammation without the pancreas ever returning to its baseline structure or function. According to the International Consensus Guidelines on Early Chronic Pancreatitis, pancreatic inflammation must last at least 6 months before it can be labelled CP. The distinction is important because, unlike AP, CP can destroy endocrine and exocrine pancreatic function, emphasising the importance of early diagnosis. As typical AP can be diagnosed by clinical symptoms plus laboratory tests, imaging is usually reserved for those with recurrent, complicated or CP. Imaging typically starts with ultrasound and more frequently with contrast-enhanced computed tomography (CECT). MRI and/or MR cholangiopancreatography can be used as a problem-solving tool to confirm indirect signs of pancreatic mass, differentiate between solid and cystic lesions, and to exclude pancreatic duct anomalies, as may occur with recurrent AP, or to visualise early signs of CP. MR cholangiopancreatography has replaced diagnostic endoscopic retrograde cholangiopancreatography (ERCP). However, ERCP, and/or endoscopic ultrasound (EUS) remain necessary for transpapillary biliary or pancreatic duct stenting and transgastric cystic fluid drainage or pancreatic tissue sampling, respectively. Finally, positron emission tomography-MRI or positron emission tomography-CT are usually reserved for complicated cases and/or to search for extra pancreatic systemic manifestations. In this article, we discuss a broad spectrum of inflammatory pancreatic disorders and the utility of various modalities in diagnosing acute and chronic pancreatitis.
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Affiliation(s)
- Nina Bastati
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Antonia Kristic
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Sarah Poetter-Lang
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Alina Messner
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Alexander Herold
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Jacqueline C Hodge
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
| | - Martin Schindl
- Department of Abdominal Surgery, Medical University of Vienna, Vienna, Austria
| | - Ahmed Ba-Ssalamah
- Department of Biomedical Imaging and Image-guided Therapy, MedicalUniversity of Vienna, Vienna, Austria
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19
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Rollon-Mayordomo A, Avellaneda-Camarena A, Gutierrez-Domingo A, Martinez-Carapeto E, Infante-Cossio P. Synchronous occurrence of IgG4-related sialadenitis and ductal carcinoma of the parotid gland: a case report. Gland Surg 2021; 10:2069-2075. [PMID: 34268092 DOI: 10.21037/gs-21-90] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2021] [Accepted: 04/09/2021] [Indexed: 11/06/2022]
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a rare chronic systemic inflammatory pathology that poses a diagnostic challenge since it can simulate malignancy when it affects a salivary gland as a mass-like lesion. Here, the authors report an unusual clinical case of a 42-year-old man who presented with a painless, slow-growing swelling located in the right parotid gland with a 12-month evolution. Based on imaging tests and open biopsy, a diagnosis of chronic parotitis was presumed and oral methylprednisolone was prescribed. Due to poor response to medication, a total parotidectomy preserving the facial nerve was performed. The final pathology described a unilateral IgG4-related sialadenitis (IgG4-RS) in the parotid gland in combination with a poorly differentiated multifocal ductal carcinoma. The postoperative course was uneventful except for a temporary facial paresis (grade III according to the House-Brackmann classification system) that resolved completely within 5 months. There were no systemic manifestations on the whole-body 18F-FDG PET/CT. Adjuvant radiotherapy was administered without complications. Twenty-four months follow-up after surgery showed no recurrence or evidence of systemic involvement. This clinical report highlights the importance of considering the synchronous occurrence of a carcinoma underlying an isolated parotid gland mass in the context of IgG4-RS, especially if there is no response to prior steroid medication.
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Affiliation(s)
- Angel Rollon-Mayordomo
- Department of Oral and Maxillofacial Surgery, Virgen Macarena University Hospital, Seville, Spain
| | - Ana Avellaneda-Camarena
- Department of Oral and Maxillofacial Surgery, Virgen Macarena University Hospital, Seville, Spain
| | | | - Elena Martinez-Carapeto
- Department of Oral and Maxillofacial Surgery, Virgen Macarena University Hospital, Seville, Spain
| | - Pedro Infante-Cossio
- Department of Surgery, School of Medicine, University of Seville, Seville, Spain
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20
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Liedtke KR, Käding C, Döring P, Bekeschus S, Glitsch AS. A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence? SAGE Open Med Case Rep 2021; 9:2050313X211016993. [PMID: 34094565 PMCID: PMC8141978 DOI: 10.1177/2050313x211016993] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/22/2020] [Accepted: 04/21/2021] [Indexed: 11/17/2022] Open
Abstract
Several chronic inflammatory diseases have been found to be a subtype of IgG4-related disease, all of which have a typical clinical and histological change, which is based in particular on an overexpression of IgG4 and subsequent fibrosis. At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4+ fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic.
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Affiliation(s)
- Kim Rouven Liedtke
- Department of General, Visceral, Thoracic and Vascular Surgery, Greifswald University Medical Centre, Greifswald, Germany.,Department of Trauma and Orthopedic Surgery, Experimental Trauma Surgery, University Medical Center Schleswig-Holstein
| | - Christoph Käding
- Department of General, Visceral, Thoracic and Vascular Surgery, Greifswald University Medical Centre, Greifswald, Germany.,Department of Urology, Greifswald University Medical Centre, Greifswald, Germany
| | - Paula Döring
- Institute of Pathology, Greifswald University Medical Centre, Greifswald, Germany
| | - Sander Bekeschus
- Centre for Innovation Competence (ZIK) plasmatis, Leibniz Institute for Plasma Science and Technology (INP Greifswald), Greifswald, Germany
| | - Anne Susann Glitsch
- Department of General, Visceral, Thoracic and Vascular Surgery, Greifswald University Medical Centre, Greifswald, Germany
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21
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Li CR, Wang JC, Lin CH, Hsu JS. Focal IgG4-related autoimmune pancreatitis mimicking pancreatic tumor. Kaohsiung J Med Sci 2021; 37:741-742. [PMID: 33728789 DOI: 10.1002/kjm2.12380] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2021] [Accepted: 02/24/2021] [Indexed: 11/11/2022] Open
Affiliation(s)
- Chuan-Ru Li
- Department of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - Jo-Ching Wang
- Department of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
| | - Chih-Hung Lin
- Department of Pathology, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan
| | - Jui-Sheng Hsu
- Department of Medical Imaging, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan
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22
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Yamamoto N, Dejima A. Rapid deterioration of diabetes mellitus with morphological changes to the pancreas. BMJ Case Rep 2021; 14:e238878. [PMID: 33541986 PMCID: PMC7868289 DOI: 10.1136/bcr-2020-238878] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/21/2021] [Indexed: 02/06/2023] Open
Affiliation(s)
- Naoki Yamamoto
- Department of Internal Medicine, Suzu General Hospital, Suzu, Ishikawa, Japan
| | - Akihiro Dejima
- Department of Internal Medicine, Suzu General Hospital, Suzu, Ishikawa, Japan
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23
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Nasser R, Gilshtein H, Mansour S, Yasin K, Borzellino G, Khuri S. Isolated Type Immunoglobulin G4 Sclerosing Cholangitis: The Misdiagnosed Cholangiocarcinoma. J Clin Med Res 2021; 13:75-81. [PMID: 33747321 PMCID: PMC7935625 DOI: 10.14740/jocmr4428] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Accepted: 01/21/2021] [Indexed: 12/13/2022] Open
Abstract
Immunoglobulin G4 sclerosing cholangitis (IgG4-SC), firstly described in 2004, is the biliary manifestation of a recently described multisystem immune-mediated disease known as IgG4-related disease. IgG4-SC is a unique and rare type of cholangitis of unknown etiology and its precise prevalence rate is still unclear. It is characterized by bile duct wall thickening and high levels of systemic serum IgG4 plasma cells. Differential diagnoses for IgG4-SC include benign (primary sclerosing cholangitis) as well as malignant (extra-hepatic cholangiocarcinoma) diseases. Discrimination between these entities is very important, due to the fact that they have different biological behaviors and different therapeutic strategies. The rare IgG4-SC subgroup with its puzzling manifestations carries a hefty diagnostic challenge for the treating physicians, and inaccurate diagnosis can lead to unnecessary morbid surgical procedures. With the paucity and relative weakness of available data in the current literature, one needs to carefully review all available parameters. A low threshold of suspicion is required to try and prevent missing IgG4-SC. IgG4-SC is highly responsive to steroid treatment, especially during the early inflammatory phase, while delay in management could lead to fibrosis and organ dysfunction. On the other hand, cholangiocarcinoma is treated by means of surgery and/or chemotherapeutic agents.
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Affiliation(s)
- Roni Nasser
- Gastroenterology and Hepatology Department, Rambam Health Care Campus, Haifa, Israel
| | - Hayim Gilshtein
- Colorectal Surgery Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
| | - Subhi Mansour
- HPB and Surgical Oncology Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
| | - Kamel Yasin
- Gastroenterology and Hepatology Department, Rambam Health Care Campus, Haifa, Israel
| | | | - Safi Khuri
- HPB and Surgical Oncology Unit, General Surgery Department, Rambam Health Care Campus, Haifa, Israel
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24
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Goyal S, Sakhuja P. Autoimmune pancreatitis: Current perspectives. INDIAN J PATHOL MICR 2021; 64:S149-S159. [PMID: 34135159 DOI: 10.4103/ijpm.ijpm_59_21] [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: 11/04/2022] Open
Abstract
Over the last two decades, our knowledge and understanding regarding the pathogenesis and biology of autoimmune pancreatitis (AIP) have improved tremendously. Type 1 AIP or IgG4-related pancreatitis (IgG4-RP) is now believed to be the prototype of the multisystemic IgG4-related disease. In view of clinical features like obstructive jaundice and mass-forming lesions in the pancreas in elderly men, type 1 AIP often mimics pancreatic cancer. IgG4-related sclerosing cholangitis concomitantly involving the extrahepatic and intrahepatic biliary tree is the most common extrapancreatic involvement seen in up to 80% of these patients, which needs to distinguish from cholangiocarcinoma. Histology is characterised by lymphoplasmacytic inflammation, abundant IgG4 positive plasma cell infiltration, storiform fibrosis and obliterative phlebitis. Apart from histology, high serum IgG4 levels, pancreatic parenchymal and duct imaging findings and other organ involvement aid in diagnosis especially in cases where definitive histology is not evident. Also, these parameters lay the foundation of various diagnostic criteria proposed over last few years. On the contrary, histology alone is the mainstay for establishing diagnosis of idiopathic duct-centric pancreatitis (IDCP) as it lacks any specific serological marker or imaging. Since both types of AIP respond dramatically to corticosteroid treatment, a biopsy is crucial to establish the preoperative diagnosis and to exclude malignancy so as to avoid unnecessary surgery. This review discusses the morphologic spectrum, treatment and prognosis of IgG4-RP and IDCP with an emphasis on approach to diagnosis with relevant histologic features, differential diagnoses and the challenges faced during biopsy interpretation.
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Affiliation(s)
- Surbhi Goyal
- Department of Pathology, GIPMER, New Delhi, India
| | - Puja Sakhuja
- Department of Pathology, GIPMER, New Delhi, India
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25
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Mitamura K, Arai-Okuda H, Yamamoto Y, Norikane T, Takami Y, Fujimoto K, Wakiya R, Ozaki H, Dobashi H, Nishiyama Y. Disease activity and response to therapy monitored by [ 18F]FDG PET/CT using volume-based indices in IgG4-related disease. EJNMMI Res 2020; 10:153. [PMID: 33296037 PMCID: PMC7726066 DOI: 10.1186/s13550-020-00743-w] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/23/2020] [Accepted: 11/25/2020] [Indexed: 12/24/2022] Open
Abstract
Purpose The efficiency of [18F]FDG PET/CT using volume-based indices was evaluated to assess the disease activity and response to therapy in patients with immunoglobulin G4-related disease (IgG4-RD). Methods A total of 17 patients with IgG4-RD were examined with [18F]FDG PET/CT before and during treatment. The lesion boundary was determined using a fixed threshold of standardized uptake value (SUV) ≥ 2.5. The highest maximum SUV (SUVmax) among all affected lesions was calculated for individual patients. We summed metabolic tumor volume (MTV) and total lesion glycolysis (TLG) of each affected lesion to generate a total MTV and total TLG. PET results were compared with those of serum IgG4 and soluble interleukin-2 receptor (sIL-2R) levels. Results The mean number of involved organs per patient was 3.8 as determined by [18F]FDG uptake. The number of involved organs, total MTV and total TLG were significantly correlated with IgG4 (P = 0.046, < 0.001, < 0.001, respectively) and sIL-2R (P < 0.001, = 0.031, 0.031, respectively). According to the clinical assessments for therapy response, all patients were classified as improved. The SUVmax, total MTV, and total TLG during therapy were all significantly lower than those before therapy (all P < 0.001). Conclusion [18F]FDG PET/CT is valuable for assessing the extent of multi-organ involvement before therapy and monitoring subsequent therapy in patients with IgG4-RD. [18F]FDG PET/CT using volumetric indices correlated with serum IgG4 and sIL-2R levels.
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Affiliation(s)
- Katsuya Mitamura
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
| | - Hanae Arai-Okuda
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
| | - Yuka Yamamoto
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan.
| | - Takashi Norikane
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
| | - Yasukage Takami
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
| | - Kengo Fujimoto
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
| | - Risa Wakiya
- Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kita-gun, Kagawa, Japan
| | - Hiroki Ozaki
- Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kita-gun, Kagawa, Japan
| | - Hiroaki Dobashi
- Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kita-gun, Kagawa, Japan
| | - Yoshihiro Nishiyama
- Department of Radiology, Faculty of Medicine, Kagawa University, 1750-1 Ikenobe, Miki-cho, Kita-gun, Kagawa, 761-0793, Japan
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Abid H, Alaoui MEHHBE, Lamrani MYA, Figuigui M, Ahmed BC, Lahmidani N, Yousfi ME, Benajah DA, Maaroufi M, Abkari ME, Ibrahimi SA, Aqodad N. [IgG4-related disease: about 3 cases]. Pan Afr Med J 2020; 36:364. [PMID: 33235641 PMCID: PMC7666702 DOI: 10.11604/pamj.2020.36.364.24835] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/05/2020] [Accepted: 07/29/2020] [Indexed: 01/11/2023] Open
Abstract
IgG4-Related disease (IgG4-RD), formerly known as IgG4-related autoimmune polyexocrinopathy, is a new condition including Plasminogen Activator Inhibitor-1 (PAI-1). It can affect different organs (central nervous system, salivary glands, thyroid, lungs, pancreas, bile ducts, liver, digestive tract, kidneys, prostate, etc.) with symptoms depending on the organ that is affected. It is more common in men older than 50 years of age. Its incidence and prevalence are poorly known because it is an uncommon disease. It is most common in Asia, accounting for only 20-30% of PAI in the Western world. Diagnosis is based on histological examination which shows dense lymphoplasmocytic infiltration in the organ affected associated with IgG4-positive plasma cells (immunohistochemistry), organ fibrosis and obliterating venulitis, all this in the context of increased serum IgG4 levels in more than 80% of cases. Patients are sensitive to corticosteroid therapy, with a high risk of relapse after discontinuation of corticosteroid therapy. This leads to the use of immunomodulators, mainly: thiopurines (azathioprine or 6-mercaptopurine), methotrexate and more recently rituximab, which can also be used as induction therapy. Given recent advances, accurate histological and clinical criteria are currently known to limit inappropriate management such as surgery. However, knowledge gaps remain concerning: pathophysiology, identification of specific biomarkers other than IgG4, natural history of the disease and long-term cancer risk assessment, performances of diagnostic tools such as endoscopic ultrasound-guided pancreatic biopsy. As well, consensual international management should be defined in the early stages of the disease and when patients develop recurrences. The purpose of this study was to report 3 cases of IgG4-Related disease on the basis of clinical and radiological criteria as well as therapeutic response.
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Affiliation(s)
- Hakima Abid
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | | | - Moulay Youssef Alaoui Lamrani
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Mouna Figuigui
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Beiba Cheikh Ahmed
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Nada Lahmidani
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Mounia El Yousfi
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Dafr-Allah Benajah
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Mustapha Maaroufi
- Service de Radiologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc.,Faculté de Médecine et de Pharmacie, Université Sidi Mohammed Ben Abdellah, Fès, Maroc
| | - Mohammed El Abkari
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Sidi Adil Ibrahimi
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Hassan II, Fès, Maroc
| | - Nourdin Aqodad
- Service d´Hépato-Gastro-Entérologie, Centre Hospitalier Universitaire Ibn Zohr, Agadir, Maroc
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Zhang R, Gao J, Zhao T, Zhang B, Wang C, Wang C, Cui L, Chen J, Fang S. A Case With IgG4-Related Spinal Pachymeningitis Causing Spinal Cord Compression. Front Neurol 2020; 11:500. [PMID: 32760335 PMCID: PMC7371922 DOI: 10.3389/fneur.2020.00500] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2019] [Accepted: 05/07/2020] [Indexed: 11/13/2022] Open
Abstract
Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by sclerosing lesions and an increased serum IgG4 level. This condition can involve any organ, but IgG4-related spinal pachymeningitis is relatively rare. In the current study, we report a case of spinal cord compression caused by IgG4-related spinal pachymeningitis. A 39-year-old man presented to us with a 15-day history of back pain and a 3-day history of dysuresia, exacerbated by weakness in the lower extremities for 2 days. Cervical magnetic resonance imaging (MRI) showed strip-shaped abnormal signals along the anterior and posterior borders of the spinal cord at the C5–T4 levels. The IgG level in cerebrospinal fluid was 718.0 mg/L. Thoracic MRI revealed strip-shaped abnormal signals with remarkable enhancement along the anterior and posterior borders of the dural sac at the T1–T6 levels. Histopathological examination confirmed IgG4-related spinal pachymeningitis. The symptoms worsened rapidly, and surgical resection of the space-occupying lesion in the vertebral canal was performed for spinal decompression. Corticosteroid therapy was administered, and the patient's motor functions were mildly improved. IgG4-related disease can manifest as spinal pachymeningitis and cause spinal cord compression. Clinicians should be aware of this rare condition, and early diagnosis, timely surgical decompression, and appropriate corticosteroid therapy should be highlighted.
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Crouser ED, Maier LA, Wilson KC, Bonham CA, Morgenthau AS, Patterson KC, Abston E, Bernstein RC, Blankstein R, Chen ES, Culver DA, Drake W, Drent M, Gerke AK, Ghobrial M, Govender P, Hamzeh N, James WE, Judson MA, Kellermeyer L, Knight S, Koth LL, Poletti V, Raman SV, Tukey MH, Westney GE. Diagnosis and Detection of Sarcoidosis. An Official American Thoracic Society Clinical Practice Guideline. Am J Respir Crit Care Med 2020; 201:e26-e51. [PMID: 32293205 PMCID: PMC7159433 DOI: 10.1164/rccm.202002-0251st] [Citation(s) in RCA: 573] [Impact Index Per Article: 114.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022] Open
Abstract
Background: The diagnosis of sarcoidosis is not standardized but is based on three major criteria: a compatible clinical presentation, finding nonnecrotizing granulomatous inflammation in one or more tissue samples, and the exclusion of alternative causes of granulomatous disease. There are no universally accepted measures to determine if each diagnostic criterion has been satisfied; therefore, the diagnosis of sarcoidosis is never fully secure. Methods: Systematic reviews and, when appropriate, meta-analyses were performed to summarize the best available evidence. The evidence was appraised using the Grading of Recommendations, Assessment, Development, and Evaluation approach and then discussed by a multidisciplinary panel. Recommendations for or against various diagnostic tests were formulated and graded after the expert panel weighed desirable and undesirable consequences, certainty of estimates, feasibility, and acceptability. Results: The clinical presentation, histopathology, and exclusion of alternative diagnoses were summarized. On the basis of the available evidence, the expert committee made 1 strong recommendation for baseline serum calcium testing, 13 conditional recommendations, and 1 best practice statement. All evidence was very low quality. Conclusions: The panel used systematic reviews of the evidence to inform clinical recommendations in favor of or against various diagnostic tests in patients with suspected or known sarcoidosis. The evidence and recommendations should be revisited as new evidence becomes available.
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Minaga K, Watanabe T, Arai Y, Shiokawa M, Hara A, Yoshikawa T, Kamata K, Yamashita K, Kudo M. Activation of interferon regulatory factor 7 in plasmacytoid dendritic cells promotes experimental autoimmune pancreatitis. J Gastroenterol 2020; 55:565-576. [PMID: 31960143 DOI: 10.1007/s00535-020-01662-2] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/06/2019] [Accepted: 01/05/2020] [Indexed: 02/04/2023]
Abstract
BACKGROUND Excessive type I IFN (IFN-I) production by plasmacytoid dendritic cells (pDCs) promotes autoimmunity. Recently, we reported that a prominent feature of both experimental autoimmune pancreatitis (AIP) and human type 1 AIP is pDC activation followed by enhanced production of IFN-I and IL-33. However, the roles played by interferon regulatory factor 7 (IRF7), a critical transcription factor for IFN-I production in pDCs, in these disorders have not been clarified. METHODS Whole and nuclear extracts were isolated from pancreatic mononuclear cells (PMNCs) from MRL/MpJ mice exhibiting AIP. Expression of phospho-IRF7 and nuclear translocation of IRF7 was examined in these extracts by immunoblotting. Pancreatic expression of IRF7 was assessed by immunofluorescence analysis in experimental AIP. Nuclear translocation of IRF7 upon exposure to neutrophil extracellular traps (NETs) was assessed in peripheral blood pDCs from type 1 AIP patients. Pancreatic IRF7 expression was examined in surgically operated specimens from type 1 AIP patients. RESULTS IRF7 activation was induced in pancreatic pDCs in experimental AIP. siRNA-mediated knockdown of IRF7 expression prevented AIP development, which was accompanied by a marked reduction in both pancreatic accumulation of pDCs and production of IFN-α and IL-33. Notably, in peripheral blood pDCs isolated from patients with type 1 AIP, nuclear translocation of IRF7 was enhanced as compared with the translocation in pDCs from healthy controls. Furthermore, IRF7-expressing pDCs were detected in the pancreas of patients with type 1 AIP. CONCLUSIONS These findings suggest that the IRF7-IFN-I-IL-33 axis activated in pDCs drives pathogenic innate immune responses associated with type 1 AIP.
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Affiliation(s)
- Kosuke Minaga
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan
| | - Tomohiro Watanabe
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan.
| | - Yasuyuki Arai
- Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Masahiro Shiokawa
- Department of Gastroenterology and Hepatology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Akane Hara
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan
| | - Tomoe Yoshikawa
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan
| | - Ken Kamata
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan
| | - Kouhei Yamashita
- Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Masatoshi Kudo
- Department of Gastroenterology and Hepatology, Kindai University Faculty of Medicine, 377-2 Ohno-Higashi, Osaka-Sayama, Osaka, 589-8511, Japan
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30
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Nakamaru K, Tomiyama T, Kobayashi S, Ikemune M, Tsukuda S, Ito T, Tanaka T, Yamaguchi T, Ando Y, Ikeura T, Fukui T, Nishio A, Takaoka M, Uchida K, Leung PSC, Gershwin ME, Okazaki K. Extracellular vesicles microRNA analysis in type 1 autoimmune pancreatitis: Increased expression of microRNA-21. Pancreatology 2020; 20:318-324. [PMID: 32147308 DOI: 10.1016/j.pan.2020.02.012] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/16/2019] [Revised: 01/24/2020] [Accepted: 02/18/2020] [Indexed: 12/11/2022]
Abstract
BACKGROUND The molecular basis of type 1 autoimmune pancreatitis (AIP) remains unclear. Recent attention on the role of extracellular vesicles microRNA (EV miRNA) in immune homeostasis has prompted us to perform an extensive miRNA screening of serum-derived EV in AIP. METHODS EV miRNA expression was analyzed using microarrays in AIP, chronic pancreatitis (CP), and healthy adult (HC) samples (n = 10 from each group). Differences in signals, > 3 or <1/3 times, represented significant differences in expression. Another cohort of AIP (n = 14), CP (n = 10), and HC (n = 10) samples of EV miRNA was analyzed using reverse-transcription polymerase chain reaction (RT-PCR). miRNA expression in pancreatic tissues was evaluated using in situ hybridization (ISH) in three additional subjects from each group. RESULTS Signals of eight miRNAs (miR-659-3p, -27a-3p, -99a-5p, -21-5p, -205-5p, -100-5p, -29c-3p, and -125b-1-3p) were significantly higher, while those of two miRNAs (miR-4252 and -5004-5p) were significantly lower in AIP than in HC. EV miR-21-5p was significantly up-regulated in AIP than in HC (P = 0.035) and CP (P = 0.048). The number of miR-21-5p positive inflammatory cells was significantly elevated in AIP than in CP (P = 0.014). CONCLUSIONS Circulating EVs exhibited altered miRNA expression patterns with elevated miR-21-5p in AIP when compared with those in HC and CP. miR-21-5p was highly expressed in pancreatic inflammatory cells in AIP. Our data suggests that miR-21-5p may be involved in the regulation of effector pathways in the pathophysiology of AIP, thus differentiating AIP from CP.
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Affiliation(s)
- Koh Nakamaru
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Takashi Tomiyama
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan.
| | - Sanshiro Kobayashi
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Manami Ikemune
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Satoshi Tsukuda
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Takashi Ito
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Toshihiro Tanaka
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Takashi Yamaguchi
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Yugo Ando
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Tsukasa Ikeura
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Toshiro Fukui
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Akiyoshi Nishio
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Makoto Takaoka
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
| | - Kazushige Uchida
- Division of Gastroenterology and Hepatology, Kochi Medical School, Kochi University, Kohasu Oko-cho, Nankoku, Kochi, 783-8505, Japan
| | - Patrick S C Leung
- Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, 451 Health Sciences Drive Suite 6510, Davis, CA, 95616, USA
| | - M E Gershwin
- Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, 451 Health Sciences Drive Suite 6510, Davis, CA, 95616, USA
| | - Kazuichi Okazaki
- Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, 2-5-1 Shinmachi, Hirakata, Osaka, 573-1010, Japan
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de las Heras Flórez S, Pérez MC, Sanz Díaz CT, Medina García JA. IgG4-related disease: a case report. ADVANCES IN LABORATORY MEDICINE 2020; 1:20190045. [PMID: 37362559 PMCID: PMC10197263 DOI: 10.1515/almed-2019-0045] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/27/2019] [Accepted: 07/27/2019] [Indexed: 06/28/2023]
Abstract
IgG4-related disease (IgG4-RD) is a condition that was first described recently, and is capable of affecting any organ of the body. Diagnosis is based on the correlation of clinical findings with histopathological findings and elevated serum IgG4. Treatment involves corticosteroids and rituximab for the most severe cases. We report the case of a symptomatic patient diagnosed of IgG4-RD whose diagnosed was guided by elevated serum IgG4 levels.
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Affiliation(s)
- Silvia de las Heras Flórez
- Nuestra Señora de la Candelaria University Hospital Ringgold standard institution - Core-Protein Unit, Carretera General del Rosario 145, 38010Santa Cruz of Tenerife, Spain
| | | | - Carmen Teresa Sanz Díaz
- Unidad Core, Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz of Tenerife, Spain
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Bavli Y, Chen BM, Roffler SR, Dobrovolskaia MA, Elnekave E, Ash S, Barenholz Y, Turjeman K. PEGylated Liposomal Methyl Prednisolone Succinate does not Induce Infusion Reactions in Patients: A Correlation Between in Vitro Immunological and in Vivo Clinical Studies. Molecules 2020; 25:molecules25030558. [PMID: 32012928 PMCID: PMC7037198 DOI: 10.3390/molecules25030558] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2019] [Revised: 01/22/2020] [Accepted: 01/23/2020] [Indexed: 12/13/2022] Open
Abstract
PEGylated nanomedicines are known to induce infusion reactions (IRs) that in some cases can be life-threatening. Herein, we report a case study in which a patient with rare mediastinal and intracardiac IgG4-related sclerosing disease received 8 treatments of intravenously administered PEGylated liposomal methylprednisolone-succinate (NSSL-MPS). Due to the ethical requirements to reduce IRs, the patient received a cocktail of premedication including low dose of steroids, acetaminophen and H2 blockers before each infusion. The treatment was well-tolerated in that IRs, complement activation, anti-PEG antibodies and accelerated blood clearance of the PEGylated drug were not detected. Prior to the clinical study, an in vitro panel of assays utilizing blood of healthy donors was used to determine the potential of a PEGylated drug to activate complement system, elicit pro-inflammatory cytokines, damage erythrocytes and affect various components of the blood coagulation system. The overall findings of the in vitro panel were negative and correlated with the results observed in the clinical phase.
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Affiliation(s)
- Yaelle Bavli
- Laboratory of Membrane and Liposome Research, Department of Biochemistry, Institute for Medical Research Israel-Canada, Hebrew University-Hadassah Medical School, Jerusalem 9112102, Israel; (Y.B.); (K.T.)
| | - Bing-Mae Chen
- Institute of Biomedical Sciences, Academia Sinica, Taipei 11529, Taiwan; (B.-M.C.); (S.R.R.)
| | - Steve R. Roffler
- Institute of Biomedical Sciences, Academia Sinica, Taipei 11529, Taiwan; (B.-M.C.); (S.R.R.)
| | - Marina A. Dobrovolskaia
- Nanotechnology Characterization Laboratory, Cancer Research Technology Program, Frederick National Laboratory for Cancer Research sponsored by the National Cancer Institute, Frederick, MD 21702, USA;
| | - Eldad Elnekave
- Davidoff Cancer Institute, Rabin Medical Center, Petach Tikva 4941492, Israel
- Correspondence: (E.E.); (Y.B.)
| | - Shifra Ash
- Rina Zaizov Pediatric Hematology Oncology Division, Schneider Children’s Medical Center of Israel, Petach Tiqva, Tel Aviv University, Tel Aviv, Israel 4920235, Israel;
| | - Yechezkel Barenholz
- Laboratory of Membrane and Liposome Research, Department of Biochemistry, Institute for Medical Research Israel-Canada, Hebrew University-Hadassah Medical School, Jerusalem 9112102, Israel; (Y.B.); (K.T.)
- Correspondence: (E.E.); (Y.B.)
| | - Keren Turjeman
- Laboratory of Membrane and Liposome Research, Department of Biochemistry, Institute for Medical Research Israel-Canada, Hebrew University-Hadassah Medical School, Jerusalem 9112102, Israel; (Y.B.); (K.T.)
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Dong P, Wang L, Li L. Bilateral orbital involvement of IgG4-related disease detected on 18F-Fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography: A Clinical Case Report. Medicine (Baltimore) 2019; 98:e18138. [PMID: 31764855 PMCID: PMC6882616 DOI: 10.1097/md.0000000000018138] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
RATIONALE IgG4-related disease (IgG4-RD) is a systemic immune-mediated fibro-inflammatory condition, which could involve multiple structures, including the pancreas, salivary glands, and lymph nodes. However, cases of IgG4-RD involving the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate are rare. PATIENT CONCERNS A 51-year-old man complaining of bilateral exophthalmos, reduced vision, and weight loss of 15 kg over 2 years presented to our department for evaluation. DIAGNOSES Based on the elevated serum IgG4 level, postoperative pathology, and the features of F-fluoro-2-deoxy-D-glucose (F-FDG) positron emission tomography/computed tomography (PET/CT), which revealed diffuse increased FDG uptake in many structures, he was diagnosed with IgG4-related disease involving the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate. INTERVENTIONS Because of the significant bilateral proptosis and exposure keratoconjunctivitis in the right eye, bilateral soft-tissue masses located in the orbits were resected, and the patient was started on oral methylprednisolone with gradual tapering. OUTCOMES The patient's symptoms gradually relieved after the operation and glucocorticoid therapy. Four months later, cranial axial CT revealed remarkable narrowing of soft-tissue masses in the bilateral orbits, and his serum IgG4 level reduced sharply. LESSONS IgG4-RD should be considered in cases of diffuse FDG uptake in the bilateral orbits, salivary glands, submandibular glands, lymph nodes, and prostate on PET/CT.
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Affiliation(s)
| | - Li Wang
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, Chengdu, PR China
| | - Lin Li
- Department of Nuclear Medicine
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35
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Abstract
Autoimmune pancreatitis (AIP) is an entity that has been recognized since 1961. Prior to the discovery of elevated serum IgG4 as a useful biomarker for its diagnosis, Dr. Yoshida in 1995 first described the entity of AIP, which in retrospect closely resembles the current concept of type 1 AIP. Since the discovery of IgG4 as a biomarker (the IgG4-era), a novel concept of IgG4-related disease (IgG4-RD) has been accepted as being comprised of two subtypes of AIP: type 1 defined as the pancreatic manifestation of IgG4-RD, and type 2 characterized by granulocytic epithelial lesions. The characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmocytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extrapancreatic manifestations of IgG4-RD (e.g., sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and steroid responsiveness. These entities can be differentiated from mimickers by a combination of serum IgG4 level, imaging features, and histopathological findings. The current first-line therapy is corticosteroids, or rituximab in high-risk patients with steroid intolerance. Although relapse rates are high, treatment of relapsed disease remains experimental.
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Affiliation(s)
- Kazuichi Okazaki
- Division of Gastroenterology and Hepatology, The Third Department of Internal Medicine, Kansai Medical University, Shinmachi, Hirakata, Osaka, 573-1197, Japan.
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36
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Fok JS, Katelaris CH. Angioedema Masqueraders. Clin Exp Allergy 2019; 49:1274-1282. [PMID: 31310036 DOI: 10.1111/cea.13463] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2019] [Revised: 06/27/2019] [Accepted: 07/05/2019] [Indexed: 12/24/2022]
Abstract
Angioedema is a common reason for referral to immunology and allergy specialists. Not all cases are in fact angioedema. There are many conditions that may mimic its appearance, resulting in misdiagnosis. This may happen when a clinician is unfamiliar with conditions resembling angioedema or when there is a low index of clinical suspicion. In this article, we explore a list of differential diagnoses based on body parts, including the lips, the limbs, periorbital tissues, the face, epiglottis and uvula, as well as the genitalia, that may pose as a masquerader even to an experienced eye.
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Affiliation(s)
- Jie Shen Fok
- Department of Respiratory Medicine, Box Hill Hospital, Melbourne, Victoria, Australia.,Faculty of Medicine, Nursing and Health Sciences, Monash University, Melbourne, Victoria, Australia
| | - Constance H Katelaris
- Immunology and Allergy Unit, Campbelltown Hospital, Campbelltown, New South Wales, Australia.,School of Medicine, Western Sydney University, Campbelltown, New South Wales, Australia
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37
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Choi JW, Choi JY, Go KH, Cheon YH, Kim JW, Lee CE, Park HO. Immunoglobulin G4-Related Aortitis of the Abdominal Aorta. THE KOREAN JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 2019; 52:239-242. [PMID: 31403030 PMCID: PMC6687050 DOI: 10.5090/kjtcs.2019.52.4.239] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/19/2018] [Revised: 12/18/2018] [Accepted: 02/22/2019] [Indexed: 12/24/2022]
Abstract
Noninfectious aortitis, inflammatory abdominal periaortitis, and idiopathic retroperitoneal fibrosis are chronic inflammatory diseases with unclear causes. Recent studies have shown that some cases of aortitis are associated with immunoglobulin G4 (IgG4)-related systemic disease. Herein, we report a case of IgG4-related aortitis (IgG4-RA) that was diagnosed after surgery. Our patient was a 46-year-old man who had experienced abdominal pain for several weeks. Preoperative evaluations revealed an area of aortitis on the infrarenal aorta. He underwent surgery, and histological examination resulted in a diagnosis of IgG4-RA.
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Affiliation(s)
- Jae Won Choi
- Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Jun Young Choi
- Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Kyung Hyuk Go
- Department of Pathology, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Yun Hong Cheon
- Division of Rheumatology, Department of Internal Medicine, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Jong Woo Kim
- Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Chung Eun Lee
- Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
| | - Hyun Oh Park
- Department of Thoracic and Cardiovascular Surgery, Gyeongsang National University Hospital, Gyeongsang National University College of Medicine, Jinju, Korea
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38
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Yang H, Li J, Wang Y, Ye S, Li J. Distribution characteristics of elevated serum immunoglobulin G 4 (IgG 4) and its relationship with IgG 4-related disease. Scand J Rheumatol 2019; 48:497-504. [PMID: 31354076 DOI: 10.1080/03009742.2019.1602882] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/24/2022]
Abstract
Objective: To elucidate the distribution characteristics of elevated serum immunoglobulin G4 (IgG4) and its relationship with immunoglobulin G4-related disease (IgG4-RD).Methods: In total, 14 522 unique patients who were tested for serum IgG4 over a 4.5 year period were enrolled. Demographic and serological characteristics of all patients, and the range of diseases and serological characteristics of patients with elevated serum IgG4, were retrospectively analysed. Organ involvement in IgG4-RD patients and the utility of serum immunoglobulin for distinguishing IgG4-RD from non-IgG4-RD were also investigated.Results: Elevated serum IgG4 (> 1.4 g/L) was observed in 1793/14 522 patients (12.3%) and primarily appeared in middle-aged and elderly men. Only 7.3% of patients with elevated serum IgG4 met the diagnostic criteria for IgG4-RD. Elevated serum IgG4 was also found in over 30 types of non-IgG4-RD. Serum IgG4 levels were higher in IgG4-RD patients with multiple-organ involvement and were positively correlated with the number of affected organs. The diagnostic utility of serum IgG4 for IgG4-RD was higher than that of the IgG4/IgG ratio and serum IgG. The optimal serum IgG4 cut-off value for diagnosing IgG4-RD was 2.06 g/L, with a sensitivity of 95.6% and specificity of 94.7%.Conclusions: Elevated serum IgG4 may be present in multiple non-IgG4-RD conditions. However, a high serum IgG4 concentration is of great value for IgG4-RD diagnosis, and there appears to be a positive relationship between serum IgG4 levels and organ involvement in patients with IgG4-RD. Examining the IgG4/IgG ratio and serum IgG does not improve the ability of IgG4-RD diagnosis.
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Affiliation(s)
- H Yang
- Department of Ophthalmology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - J Li
- Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Y Wang
- Department of Ophthalmology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - S Ye
- Rheumatology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - J Li
- Department of Ophthalmology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
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Abstract
Diagnosis of the inflammatory aortopathies and importantly, their distinction in the later stages of disease from genetically mediated or acquired (degenerative) aortopathy remains a challenging clinical problem. Historically, the diagnosis of inflammatory aortopathy has required tissue sampling and pathological assessment. Although histological diagnosis remains an important diagnostic criterion, the ability to obtain sufficient tissue samples is problematic and requires invasive approaches that pose important risk. Continuing refinement in the capabilities of multimodality imaging, including ultrasound, computed tomography, magnetic resonance imaging, and positron emission tomography provides important insights into the broad spectrum of disease which comprise the inflammatory aortopathies. This review examines the current and emerging role of multimodality imaging in the evaluation of aortitis.
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Affiliation(s)
- John P Bois
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN
| | - Vidhu Anand
- Department of Cardiovascular Medicine, Mayo Clinic, Rochester, MN
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40
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Sugimoto M, Takagi T, Suzuki R, Konno N, Asama H, Sato Y, Irie H, Watanabe K, Nakamura J, Kikuchi H, Takasumi M, Hashimoto M, Hikichi T, Ohira H. Present state of endoscopic ultrasonography-guided fine needle aspiration for the diagnosis of autoimmune pancreatitis type 1. World J Meta-Anal 2019; 7:218-223. [DOI: 10.13105/wjma.v7.i5.218] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/16/2019] [Revised: 04/27/2019] [Accepted: 05/01/2019] [Indexed: 02/06/2023] Open
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41
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Olson E, Perelman A, Birk JW. Acute management of pancreatitis: the key to best outcomes. Postgrad Med J 2019; 95:328-333. [PMID: 31123175 DOI: 10.1136/postgradmedj-2018-136034] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2018] [Revised: 10/11/2018] [Accepted: 04/25/2019] [Indexed: 12/17/2022]
Abstract
Acute pancreatitis (AP) accounts for over 230 000 US and 28 000 UK hospital admissions annually. Abdominal pain is the most common presenting symptom in AP but may not reflect severity. The clinical challenge is identifying the 20% of patients in whom AP will be severe. We summarise the common aetiologies, the risk stratification strategies including the simplified Bedside Index for Severity in Acute Pancreatitis, acute management approaches in the initial presentation setting, conditions for using advance imaging and opinions on antibiotic use. Some warning signs of impending complications are also discussed.
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Affiliation(s)
- Erik Olson
- Department of Gastroenterology and Hepatology, Rochester General Hospital, Rochester, New York, USA
| | | | - John W Birk
- Gastroenterology-Hepatology, University of Connecticut School of Medicine, Farmington, Connecticut, USA
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42
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Vahabzadeh-Hagh AM, Ramirez E. Chronic Asymptomatic Parotid Mass as a Focal Manifestation of Immunoglobulin G4-Related Disease. EAR, NOSE & THROAT JOURNAL 2019; 99:365-366. [PMID: 31088308 DOI: 10.1177/0145561319840533] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Affiliation(s)
- Andrew M Vahabzadeh-Hagh
- Department of Head and Neck Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA, USA.,Department of Surgery - Division of Otolaryngology, University of California, San Diego, CA, USA
| | - Eddie Ramirez
- Department of Head and Neck Surgery, UCLA David Geffen School of Medicine, Los Angeles, CA, USA
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43
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Bowman AW, Bolan CW. MRI evaluation of pancreatic ductal adenocarcinoma: diagnosis, mimics, and staging. Abdom Radiol (NY) 2019; 44:936-949. [PMID: 29967986 DOI: 10.1007/s00261-018-1686-x] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
The radiologist's role in the evaluation of pancreatic ductal adenocarcinoma remains critical in the management of this deadly disease. Imaging plays a vital role in the diagnosis and staging of pancreatic cancer. Although CT is more commonly used for staging pancreatic cancer, MR is increasingly playing an important role in this regard. In our institution, all pancreatic malignancies undergo staging with MRI. In this pictoral essay, we illustrate the MR imaging features of pancreatic ductal adenocarcinoma and its mimics, and we also discuss pearls and pitfalls in MR staging of pancreatic carcinoma.
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Affiliation(s)
- Andrew W Bowman
- Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.
| | - Candice W Bolan
- Department of Radiology, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA
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44
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Pagliari D, Cianci R, Rigante D. Autoimmune pancreatitis in children: The impact of immune system in a challenging disease. Autoimmun Rev 2019; 18:209-210. [PMID: 30572132 DOI: 10.1016/j.autrev.2018.09.006] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/20/2018] [Accepted: 09/26/2018] [Indexed: 01/13/2023]
Affiliation(s)
- Danilo Pagliari
- Institute of Internal Medicine and Gastroenterology, Pancreatic Unit, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
| | - Rossella Cianci
- Institute of Internal Medicine, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy
| | - Donato Rigante
- Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Università Cattolica Sacro Cuore, Rome, Italy
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45
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Abstract
Autoimmune pancreatitis (AIP) is a rare disorder characterized by prompt clinical response to corticosteroids. Lost tolerance to a variety of pancreatic antigens and subsequent development of autoantibodies are presumably involved in the initiation of AIP. Even pediatric patients have been reported with features of AIP, and awareness of this disorder is increasing among different clinicians. The terms lymphoplasmacytic sclerosing pancreatitis and idiopathic duct-centric pancreatitis refer to the different histologic patterns of AIP, named type 1 and type 2, respectively. A combination of serologic, radiologic, and histologic investigations is needed to assess diagnosis of AIP and rule out neoplastic disorders. In addition, type 1 AIP can be distinguished by raised levels of serum immunoglobulin G4 and should be considered as part of systemic immunoglobulin G4-related disease. Conversely, type 2 AIP is frequently reported in younger patients and has less clear immune-mediated pathogenetic mechanisms. The natural history of pediatric AIP is obscure, and the diagnostic usefulness of different autoimmune abnormalities found in adults with AIP is limited for children. Tips to manage pediatric patients with AIP have been recently drafted through a set of recommendation statements. This review describes the current data about AIP and the pathogenic contribution of specific autoantibodies expressly in the pediatric population.
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Affiliation(s)
- Danilo Pagliari
- From the Institutes of Internal Medicine and Gastroenterology and Pancreatic Unit
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46
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Mejías Manzano MDLÁ, Trigo Salado C, Serrano Jiménez M, Parada Blázquez MJ, Leo Carnerero E. IgG4-related sclerosing mesenteritis, a rare condition that causes abdominal pain. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2018; 110:201-203. [PMID: 29271222 DOI: 10.17235/reed.2017.5228/2017] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
The identification of IgG4-related disease as a distinct immune-mediated condition encompassing disorders that were traditionally seen as idiopathic has been a revolution in the diagnostic and therapeutic algorithm in several medical fields. This condition usually involves multiple organs (isolated organ involvement is uncommon except in the pancreas) with characteristic histopathological findings. We report a case that was assessed due to abdominal pain and subsequently diagnosed with IgG4-related sclerosing mesenteritis. A comprehensive work-up of the case ruled out other conditions and a diagnosis of IgG4-related sclerosing mesenteritis was made according to radiographic and histopathological criteria.
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Affiliation(s)
| | - Claudio Trigo Salado
- Unidad de Gestión Clínica de Aparato Digestivo, Hospital Universitario Virgen del Rocío, España
| | | | | | - Eduardo Leo Carnerero
- Unidad de Gestión Clínica de Aparato Digestivo, Hospital Universitario Virgen del Rocío, España
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47
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Goodchild G, Pereira SP, Webster G. Immunoglobulin G4-related sclerosing cholangitis. Korean J Intern Med 2018; 33:841-850. [PMID: 30045615 PMCID: PMC6129623 DOI: 10.3904/kjim.2018.018] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/09/2018] [Accepted: 02/06/2018] [Indexed: 12/22/2022] Open
Abstract
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition of which IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC, namely, primary sclerosing cholangitis and cholangiocarcinoma, outline diagnostic criteria for IgG4-SC, provide insight into possible pathophysiological mechanisms underlying the disease and discuss short and long-term management options of this recently described disease.
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Affiliation(s)
- George Goodchild
- Department of Gastroenterology, University College Hospital, London, UK
| | | | - George Webster
- Department of Gastroenterology, University College Hospital, London, UK
- Correspondence to George Webster, M.D. Department of Gastroenterology, University College Hospital, 250 Euston Rd, London NW1 2PG, UK Tel: +44-2034567890 Fax: +44-2034479218 E-mail:
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48
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Wuesthoff C, Allende A, Patel N. IgG4 disease of the ear: Report and review. SAGE Open Med Case Rep 2018; 6:2050313X18791428. [PMID: 30116527 PMCID: PMC6088471 DOI: 10.1177/2050313x18791428] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2018] [Accepted: 07/05/2018] [Indexed: 12/24/2022] Open
Abstract
In recent years, an immune-mediated disorder involving IgG4 has been described, which targets multiple organs and explains a number of disorders previously regarded as “idiopathic” or of unknown origin. Furthermore, the discovery of IgG4-related disease (IgG4-RD) has placed a number of pathologies within its spectrum, linking symptoms and conditions formerly considered isolated. Reports of the manifestations of IgG4-RD in the head and neck are scarce. Otological manifestations have been reported, but only a handful of cases are available in the literature. This is the first report of recalcitrant serous otitis media secondary to IgG4-RD, confirmed by immunohistopathology. A case of IgG4-RD of the middle ear is presented, manifesting itself as recalcitrant serous otitis media. The case is presented from an otolaryngological and histopathological perspective and briefly reviews this rare disorder. The importance of the awareness of IgG4-RD resides mainly in the fact that it is a treatable condition. This can potentially improve the quality of life of a number of patients, some of whom may not have had a clear diagnosis. A favorable response to glucocorticoids has been reported. In cases of persistent symptoms, immunosuppressive therapy has been used with success.
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Affiliation(s)
- Carolina Wuesthoff
- Deafness Research, Kolling Institute of Medical Research and Department of Otolaryngology, Head and Neck Surgery, Royal North Shore Hospital, Sydney, NSW, Australia
| | - Alexandra Allende
- Department of Histopathology, Douglass Hanly Moir Pathology, Sydney, NSW, Australia.,Department of Clinical Medicine, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, NSW, Australia
| | - Nirmal Patel
- Deafness Research, Kolling Institute of Medical Research and Department of Otolaryngology, Head and Neck Surgery, Royal North Shore Hospital, Sydney, NSW, Australia.,Discipline of Surgery, Sydney Medical School, The University of Sydney, Sydney, NSW, Australia.,Department of ORL-Head and Neck Surgery, Macquarie University, Sydney, NSW, Australia
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49
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Yamamoto K, Itoi T, Sofuni A, Tsuchiya T, Tsuji S, Tanaka R, Tonozuka R, Honjo M, Mukai S, Kamada K, Fujita M, Asai Y, Matsunami Y, Nagakawa Y. The Role of Endoscopic Ultrasound-guided Drainage for Autoimmune Pancreatitis-associated Pancreatic Cysts: A Report of Five Cases and a Literature Review. Intern Med 2018; 57:1523-1531. [PMID: 29434161 PMCID: PMC6028677 DOI: 10.2169/internalmedicine.9779-17] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/07/2017] [Accepted: 09/26/2017] [Indexed: 12/29/2022] Open
Abstract
Objective Autoimmune pancreatitis (AIP) has been recognized as a benign disease, which that shows a prompt response to corticosteroid treatment (CST). It was previously believed to not be associated with cyst formation; however, a few cases of AIP-associated pancreatic cyst (PC) have been reported. Some cases were reported to have been effectively treated by CST, while others were refractory to CST. Many of the patients received interventional treatment. Until now, there has been no consensus on the therapeutic strategies for AIP-associated PC. The aim of the present study is to describe a therapeutic strategy for this condition. Methods We conducted a retrospective study of 5 cases of AIP-associated PC that were treated by endoscopic ultrasonography-guided pancreatic fluid collection drainage (ESPD) or CST at Tokyo Medical University Hospital between March 2012 and October 2016, analyzed the therapeutic outcomes, and performed a literature review. Results The initial treatments included CST (n=2) and ESPD (n=3). All of the PCs disappeared after treatment In 1 of the patients who received CST case and 3 of the patients who received ESPD; however, the PC did not disappear in one of the patients who received CST (corticosteroid maintenance therapy), even after the dose of corticosteroids was increased; ESPD was eventually performed and the PC disappeared. There were no procedure-related complaints. Conclusion We propose that CST be administered as the first-line treatment for AIP-associated PC, particularly in cases of PC without a history of CST. However, ESPD can be applied to treat cases of corticosteroid refractory PC.
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Affiliation(s)
- Kenjiro Yamamoto
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Atsushi Sofuni
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Takayoshi Tsuchiya
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Shujiro Tsuji
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Reina Tanaka
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Ryosuke Tonozuka
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Mitsuyoshi Honjo
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Shuntaro Mukai
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Kentaro Kamada
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Mitsuru Fujita
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Yasutsugu Asai
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
| | - Yukitoshi Matsunami
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Japan
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50
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Abstract
RATIONALE Immunoglobulin (Ig)G4-related pseudotumors of the liver are very rare diseases that are difficult to distinguish from malignant tumors. They can be usually improved by steroid therapy. Actinomycosis is a chronic, suppurative, granulomatous infection, for which immune suppression is a predisposing factor. It can also mimic malignant tumors. PATIENT CONCERNS A 67-year-old man presented with mild abdominal discomfort and a 5-kg weight loss for 3 months. Initially, he visited another hospital and was treated with antibiotics under the assumption of a liver abscess. Symptom was not resolved. DIAGNOSES He diagnosed as having an IgG4-related pseudotumor of the right lobe of the liver after liver biopsy. Despite 2 months of steroid therapy, the liver mass was aggravated and invaded the right lung, as observed on follow-up computed tomography scan. INTERVENTIONS We performed en bloc resection of the tumor under the assumption that it was a malignant tumor. OUTCOMES The pathology of the tumor was revealed as actinomycotic colonies and IgG4-positive plasma cells of the liver. He recovered well and was discharged with ursodeoxycholic acid tablet for 14 days. After 3 months, he underwent postoperative follow-up CT and there was no remarkable finding in remnant left hepatic lobe. LESSONS Hepatic actinomycosis and IgG4-related pseudotumors of the liver are both difficult to diagnose. As in our patient, combined diseases are more difficult to diagnose and to determine the optimal treatment. Since immunosuppression therapy of autoimmune diseases can cause and aggravate infection, management must be approached carefully. We can learn that various possibilities must be considered before diagnosing and treating a hepatic mass.
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Affiliation(s)
| | - Hyung Sun Kim
- Pancreatobiliary Cancer Clinic, Department of Surgery
| | | | - Dong Ki Lee
- Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University, Seoul, Korea
| | - Jin Hong Lim
- Pancreatobiliary Cancer Clinic, Department of Surgery
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