Perivascular fibrosis and IgG4-related disease: a case report

• Main Authors: S. Monti, G. Crepaldi, A. Peri, A. Pietrabissa, P. Morbini, F. Bobbio-Pallavicini, C. Montecucco, R. Caporali
• Format: Article
• Language: English
• Published: PAGEPress Publications 2014-11-01
• Series: Reumatismo
• Subjects: Immunoglobulin G4-related disease, Perivascular fibrosis, Retroperitoneal fibrosis, Aortic aneurysm.
• Online Access: http://www.reumatismo.org/index.php/reuma/article/view/753

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.