Periaortitis, Coronary Vasculitis and Retro-Orbital Pseudotumor in a Patient with Immunoglobulin G4-Related Sclerosing Vasculitis
Burak BİLGİN, Yakup YEŞİLKAYA, Burcu TÜRKER, Özkan VARAN, Öznur AYDIN, Metin IŞIK, Levent KILIÇ, İsmail DOĞAN, Başak BOLAYIR, Sedat KİRAZ, Enver ATALAR, Mustafa CENGİZ
Department of Internal Medicine, Medical Faculty of Hacettepe University, Division of Rheumatology, Ankara, Turkey
Keywords: IgG4-related sclerosing vasculitis; inflammatory pseudotumor
Abstract
Immunoglobulin G4 (IgG4)-related sclerosing vasculitis is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Serum IgG4 level and immunostaining with anti-IgG4 antibody are useful for the diagnosis. In this article, a 61-year-old male patient who was admitted with the complaints of dyspnea, diffuse edema and chest pain and presented with increased pericardial fluid and serum IgG4 level was reported. Thoracoabdominal computed tomography (CT) scan demonstrated a soft tissue mass starting at arcus aorta and surrounding thoracal and abdominal aorta. After biopsy was obtained, the patient was diagnosed with IgG4-related sclerosing vasculitis.