The many faces of IgG4-related disease: report of a case with inaugural recurrent aortic aneurism ruptures and literature review

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-yea...

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Bibliographic Details
Published in:Rheumatology international 2018-08, Vol.38 (8), p.1565-1570
Main Authors: Luís, Mariana, Brites, Luísa, Fernandes, Bruno, Jesus, Diogo, Santiago, Tânia, Serra, Sara, Rovisco, João, Carvalho, Lina, da Silva, José António P., Malcata, Armando
Format: Article
Language:English
Subjects:
Abdomen
Aneurysms
Biopsy
Cases with a Message
Clinical medicine
Coronary vessels
Histopathology
Literature reviews
Medicine
Medicine & Public Health
Rheumatology
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Summary: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.
ISSN:0172-8172
1437-160X
DOI:10.1007/s00296-018-4046-9