A case of incidentally detected IgG4-related sclerosing disease involving inflammatory abdominal aortic aneurysm and autoimmune pancreatitis

Vol.20 No.3

Case Report

A case of incidentally detected IgG4-related sclerosing disease involving inflammatory abdominal aortic aneurysm and autoimmune pancreatitis

Authors

Yasunori Matsuki¹ , Kimiya Sato² , Akira Fujikawa³ , Yukishige Kyoto³ , Hiroshi Hashimoto⁴ , Yukiya Hakozaki¹

Department of Medicine, Japan Self-Defense Force Central Hospital, 1-2-24 Ikejiri, Setagaya Tokyo, 154-8532, Japan
Department of Pathology, Japan Self-Defense Force Central Hospital, Setagaya, Tokyo, Japan
Department of Radiology, Japan Self-Defense Force Central Hospital, Setagaya, Tokyo, Japan
Department of Respiratory Surgery, Japan Self-Defense Force Central Hospital, Setagaya, Tokyo, Japan

Received:

7 August 2009

Accepted:

12 January 2010

Published online:

16 February 2010

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Abstract

A 59-year-old asymptomatic man was incidentally found to have a periaortic mass and an elevated serum amylase level during his medical check-up. Additional findings, such as infiltration of immunoglobulin G4 (IgG4)-producing plasma cells in the mass lesion, elevation of serum IgG4 (1000 mg/dl), and pancreatic duct narrowing as evidenced on a magnetic resonance cholangiopancreatography scan, confirmed the diagnosis as retroperitoneal fibrosis complicated with autoimmune pancreatitis. The patient responded favorably to steroid treatment before the appearance of symptoms.

Key words

Corticosteroid - Multifocal fibrosclerosis - Retroperitoneal fibrosis