Constrictive pericarditis as first presentation of IgG4-related disease: Pitfalls and pearls

IgG4-related disease (IgG4-RD) is an under-recognised multisystem inflammatory disorder that has several typical manifestations. Cardiac manifestations of IgG4-RD are well documented; however, they do not feature in the definition or diagnosis of IgG4-RD according to a recent consensus statement. The most well-recognised cardiac manifestation of IgG4-RD, pericardial disease, is outlined in this case report as the initial presenting pathology. In the present case, the diagnosis was delayed due to the relative obscurity of cardiac manifestations of IgG4-RD and exposed the patient to the risks of pericardiectomy.

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Publication Details

Journal
Modern Rheumatology Case Reports
Published
2024-12-23
DOI
https://doi.org/10.1093/mrcr/rxae089
Primary Topic
IgG4-Related and Inflammatory Diseases
Type
article
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article

Constrictive pericarditis as first presentation of IgG4-related disease: Pitfalls and pearls

John Yiannikas, Timothy N. Kwan, Gemma Kwan
Modern Rheumatology Case Reports
IgG4-Related and Inflammatory Diseases
article

Constrictive pericarditis as first presentation of IgG4-related disease: Pitfalls and pearls

John Yiannikas, Timothy N. Kwan, Gemma Kwan
article en

Abstract

IgG4-related disease (IgG4-RD) is an under-recognised multisystem inflammatory disorder that has several typical manifestations. Cardiac manifestations of IgG4-RD are well documented; however, they do not feature in the definition or diagnosis of IgG4-RD according to a recent consensus statement. The most well-recognised cardiac manifestation of IgG4-RD, pericardial disease, is outlined in this case report as the initial presenting pathology. In the present case, the diagnosis was delayed due to the relative obscurity of cardiac manifestations of IgG4-RD and exposed the patient to the risks of pericardiectomy.

Modern Rheumatology Case ReportsVol. 9(2)
The University of Sydney (AU), Concord Hospital (US), Concord Repatriation General Hospital (AU)
Good health and well-being
Openalex Percentile: Top 59%
IgG4-Related and Inflammatory Diseases
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