-Case Reports-

Autoimmune Pancreatitis Associated with Idiopathic Retroperitoneal Fibrosis: A Case Report

Takayuki Aimoto¹, Eiji Uchida¹, Yoshiharu Nakamura¹, Akira Katsuno¹, Kazumitsu Chou¹, Takashi Tajiri¹ and Zenya Naito²

¹Surgery for Organ Function and Biological Regulation, Nippon Medical School Graduate School of Medicine
²Second Department of Pathology, Nippon Medical School

A 69-year-old man presented with obstructive jaundice and dark urine. Contrast-enhanced computed tomography revealed an enlarged pancreas with homogenous enhancement. Endoscopic retrograde pancreatography demonstrated short-segmental, irregular narrowing of the main pancreatic duct. The patient underwent exploratory laparotomy and needle biopsies of the pancreas, which showed marked fibrotic change with lymphocyte infiltration. These clinicopathologic findings suggested autoimmune pancreatitis. Four years later, computed tomography demonstrated marked periaortic soft tissue surrounding a calcified infrarenal abdominal aorta compatible with retroperitoneal fibrosis. We diagnosed retroperitoneal fibrosis with noncontiguous pancreatic fibrosis. This patient responded well to corticosteroid treatment. Autoimmune pancreatitis associated with idiopathic retroperitoneal fibrosis seems to be extremely rare, and to our knowledge, only a few cases have been reported.

J Nippon Med Sch 2006; 73: 235-239

Keywords
autoimmune pancreatitis, idiopathic retroperitoneal fibrosis, multifocal idiopathic fibrosclerosis, IgG4, steroid therapy

Correspondence to
Takayuki Aimoto, MD, Department of Surgery, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
aimoto@maple.ocn.ne.jp

Received, April 17, 2006.
Accepted, May 16, 2006.