Case Reports
Vol. 66 No. 3 (2014)
https://doi.org/10.4081/reumatismo.2014.753

Perivascular fibrosis and IgG4-related disease: a case report

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Received: 23 May 2014
Published: 6 November 2014
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Immunoglobulin G4-related disease, Perivascular fibrosis, Retroperitoneal fibrosis, Aortic aneurysm.
Rheumatology

Authors

S. Monti
saram86@libero.it
U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
G. Crepaldi
U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
A. Peri
U.O. Chirurgia Generale, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
A. Pietrabissa
U.O. Chirurgia Generale, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
P. Morbini
U.O. Anatomia Patologica, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
F. Bobbio-Pallavicini
U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
C. Montecucco
U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
R. Caporali
U.O. Reumatologia, Università di Pavia, IRCCS Fondazione Policlinico S. Matteo, Pavia, Italy.
Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibroinflammatory condition which can potentially involve any organ. Some characteristic histopathologic features with lymphoplasmacytic infiltrate, an increased number of IgG4+ cells, storiform fibrosis and obliterative phlebitis are the mainstay for diagnosis. Serum IgG4 levels often increase. We report the case of a patient with perivascular fibrotic lesions involving the aortic arch and the splenic hilum, with a surgical biopsy-proven diagnosis of IgG4-related disease. The patient is now undergoing a low-dose corticosteroid maintenance therapy without evidence of new localizations of the disease. This case highlights the need for increasing awareness and recognition of this new, emerging clinical condition.

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1.
Perivascular fibrosis and IgG4-related disease: a case report. Reumatismo [Internet]. 2014 Nov. 6 [cited 2026 Sep. 13];66(3):245-8. Available from: https://www.reumatismo.org/reuma/article/view/reumatismo.2014.753

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