1. Thoracic involvement in IgG4-related disease in a UK-based patient cohort.
- Author
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Corcoran JP, Culver EL, Anstey RM, Talwar A, Manganis CD, Cargill TN, Hallifax RJ, Psallidas I, Rahman NM, and Barnes E
- Subjects
- Adult, Aged, Aged, 80 and over, Autoimmune Diseases complications, Autoimmune Diseases physiopathology, Female, Fibrosis, Granuloma, Plasma Cell etiology, Granuloma, Plasma Cell physiopathology, Humans, Lung Diseases, Interstitial etiology, Lung Diseases, Interstitial physiopathology, Lymphadenopathy etiology, Lymphadenopathy physiopathology, Male, Mediastinitis etiology, Mediastinitis physiopathology, Mediastinum pathology, Middle Aged, Pleural Diseases etiology, Pleural Diseases physiopathology, United Kingdom, Young Adult, Autoimmune Diseases immunology, Granuloma, Plasma Cell immunology, Immunoglobulin G immunology, Lung Diseases, Interstitial immunology, Lymphadenopathy immunology, Mediastinitis immunology, Pleural Diseases immunology
- Abstract
IgG4-related disease (IgG4-RD) is a multi-system fibro-inflammatory disorder with classical histopathological findings, often in the context of elevated serum IgG4 levels. The thoracic manifestations of IgG4-RD are numerous and can mimic several common and better known conditions. The objective of this study was to outline the frequency and nature of thoracic involvement in a prospective cohort of IgG4-RD patients who met defined diagnostic criteria. Over 40% of IgG4-RD patients had clinicoradiological and/or histological evidence of thoracic involvement, predominantly mediastinal lymphadenopathy, the majority associated with multi-system disease outside the chest. Thoracic involvement was associated with a higher serum IgG4 level, potentially representing greater disease activity or spread. Our data highlight the diverse nature of thoracic IgG4-RD, and the importance of knowledge and recognition of the condition among respiratory physicians who are likely to encounter this disease entity on an increasing basis., (Copyright © 2017 Elsevier Ltd. All rights reserved.)
- Published
- 2017
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