Thoracic involvement in IgG4-related disease in a UK-based patient cohort

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 t...

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Main Authors: Corcoran, J, Culver, E, Anstey, R, Talwar, A, Manganis, C, Cargill, T, Hallifax, R, Psallidas, I, Rahman, N, Barnes, E
Format: Journal article
Published: Elsevier 2017
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author Corcoran, J
Culver, E
Anstey, R
Talwar, A
Manganis, C
Cargill, T
Hallifax, R
Psallidas, I
Rahman, N
Barnes, E
author_facet Corcoran, J
Culver, E
Anstey, R
Talwar, A
Manganis, C
Cargill, T
Hallifax, R
Psallidas, I
Rahman, N
Barnes, E
author_sort Corcoran, J
collection OXFORD
description 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.
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spelling oxford-uuid:195ccaf8-47fb-4a54-b329-8b517f449d752022-03-26T10:48:38ZThoracic involvement in IgG4-related disease in a UK-based patient cohortJournal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:195ccaf8-47fb-4a54-b329-8b517f449d75Symplectic Elements at OxfordElsevier2017Corcoran, JCulver, EAnstey, RTalwar, AManganis, CCargill, THallifax, RPsallidas, IRahman, NBarnes, EIgG4-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.
spellingShingle Corcoran, J
Culver, E
Anstey, R
Talwar, A
Manganis, C
Cargill, T
Hallifax, R
Psallidas, I
Rahman, N
Barnes, E
Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title_full Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title_fullStr Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title_full_unstemmed Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title_short Thoracic involvement in IgG4-related disease in a UK-based patient cohort
title_sort thoracic involvement in igg4 related disease in a uk based patient cohort
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