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12.05.2018 | Cases with a Message

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

verfasst von: Mariana Luís, Luísa Brites, Bruno Fernandes, Diogo Jesus, Tânia Santiago, Sara Serra, João Rovisco, Lina Carvalho, José António P. da Silva, Armando Malcata

Erschienen in: Rheumatology International | Ausgabe 8/2018

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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.

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Titel: The many faces of IgG4-related disease: report of a case with inaugural recurrent aortic aneurism ruptures and literature review
verfasst von: Mariana Luís
Luísa Brites
Bruno Fernandes
Diogo Jesus
Tânia Santiago
Sara Serra
João Rovisco
Lina Carvalho
José António P. da Silva
Armando Malcata
Publikationsdatum: 12.05.2018
Verlag: Springer Berlin Heidelberg
Erschienen in: Rheumatology International / Ausgabe 8/2018
Print ISSN: 0172-8172
Elektronische ISSN: 1437-160X
DOI: https://doi.org/10.1007/s00296-018-4046-9

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