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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.

Original publication

DOI

10.1016/j.rmed.2017.10.005

Type

Journal article

Journal

Respir Med

Publication Date

11/2017

Volume

132

Pages

117 - 121

Keywords

IgG4-related disease, Adult, Aged, Aged, 80 and over, Autoimmune Diseases, Female, Fibrosis, Granuloma, Plasma Cell, Humans, Immunoglobulin G, Lung Diseases, Interstitial, Lymphadenopathy, Male, Mediastinitis, Mediastinum, Middle Aged, Pleural Diseases, United Kingdom, Young Adult