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Received: March 9, 2011 Accepted: July 15, 2011 Correspondence: Metin Isik, M.D. Hacettepe Universitesi Tip Fakultesi Ic Hastaliklari Anabilim Dali, Romatoloji Bilim Dali, 06100 Sihhiye, Ankara, Turkey. Tel: +90 312 508 46 03 e-mail: metin1721978@yahoo.com ©2012 Turkish League Against Rheumatism. All rights reserved. Immunoglobulin G4 (IgG4) iliskili sklerozan vaskulit, cesitli organlarda yaygin IgG4-pozitif plazma hucreleri ve T-lenfosit infiltrasyonu ile karakterize bir hastaliktir. Serum IgG4 duzeyleri ve anti-IgG4 antikor immun boyamalari tanida kullanislidir. Bu makalede, dispne, yaygin odem ve gogus agrisi yakinmasiyla basvuran ve perikardiyal sivi ve serum IgG4 duzeyinde artis gorulen 61 yasinda bir erkek hasta sunuldu. Torakoabdominal bilgisayarli tomografi (BT) taramasinda aort arktan baslayan ve torakal ve abdominal aortu saran bir yumusak doku kitlesi saptandi. Biyopsi yapildiktan sonra, hastaya IgG4 iliskili sklerozan vaskulit tanisi kondu. Anahtar sozcukler: IgG4 iliskili sklerozan vaskulit; inflamatuvar psodotumor. 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.

Periaortitis, Coronary Vasculitis and Retro-Orbital Pseudotumor in a Patient with Immunoglobulin G4-Related Sclerosing Vasculitis