The Many Faces of IgG4-related Disease: Report of a Case with Inaugural Recurrent Aortic Aneurism Ruptures and Literature Review

Overview

Journal Rheumatol Int

Specialty Rheumatology

Date 2018 May 14

PMID 29754327

Citations 3

Authors

Mariana Luis

Luisa Brites

Bruno Fernandes

Diogo Jesus

Tania Santiago

Sara Serra

Joao Rovisco

Lina Carvalho

Jose Antonio P da Silva

Armando Malcata

Affiliations

Soon will be listed here.

Abstract

Vascular involvement in IgG4-related disease (IgG4-RD), is a well-recognized feature and large vessel commitment, especially the aorta, can be the only manifestation of the disease. Being a newly recognized disease, its diagnosis and workup still represents a challenge in clinical practice. A 47-year-old-man with two aortic aneurysms ruptures, one at abdominal and the other at thoracic level, was referred to our rheumatology department. The initial analysis of the surgical specimen obtained 3 years earlier revealed a nonspecific aortitis. Re-evaluation of the biopsy with immunohistology now demonstrated the presence of IgG4 deposits. Evidence-based recommendations regarding diagnosis, treatment and follow-up of IgG4-related large-vessel involvement are lacking. In this particular case, histopathology were crucial. The authors review and discuss vascular involvement in IgG4-RD and respective treatment options.

Citing Articles

A rare case of IgG4-related aortitis in the thoracic aorta mimicking an intramural hematoma: navigating the diagnostic labyrinth.

Alemany V, Fortier J, Gupta H, Zaider A, Grau J, Burns P J Cardiothorac Surg. 2024; 19(1):599.

PMID: 39379977 PMC: 11460049. DOI: 10.1186/s13019-024-03026-w.

Adventitial matrix metalloproteinase production and distribution of immunoglobulin G4-related abdominal aortic aneurysms.

Kasashima S, Kawashima A, Kasashima F, Matsumoto Y, Yamamoto Y, Ozaki S JVS Vasc Sci. 2021; 1:151-165.

PMID: 34617043 PMC: 8489202. DOI: 10.1016/j.jvssci.2020.06.001.

IgG4 aortitis of the ascending thoracic aorta: A case report and literature review.

Shilagani C, Lansman S, Gilet A, Flusberg M J Radiol Case Rep. 2021; 15(5):1-9.

PMID: 34276873 PMC: 8253153. DOI: 10.3941/jrcr.v15i5.3985.

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