Brief Articles
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World J Gastroenterol. May 21, 2009; 15(19): 2357-2360
Published online May 21, 2009. doi: 10.3748/wjg.15.2357
Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis
Terumi Kamisawa, Kensuke Takuma, Hajime Anjiki, Naoto Egawa, Masanao Kurata, Goro Honda, Kouji Tsuruta
Terumi Kamisawa, Kensuke Takuma, Hajime Anjiki, Naoto Egawa, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan
Masanao Kurata, Goro Honda, Kouji Tsuruta, Department of Surgery, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan
Author contributions: Kamisawa T designed the study; Takuma K, Anjiki H, Egawa N, Kurata M, Honda G, and Tsuruta K gathered and analyzed the data; Kamisawa T wrote the paper.
Correspondence to: Terumi Kamisawa, MD, PhD, Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo 113-8677, Japan. kamisawa@cick.jp
Telephone: +81-3-38232101
Fax: +81-3-38241552
Received: February 4, 2009
Revised: April 10, 2009
Accepted: April 17, 2009
Published online: May 21, 2009
Abstract

AIM: To clarify the characteristic features of biliary lesions in patients with autoimmune pancreatitis (AIP) and compare them with those of primary sclerosing cholangitis (PSC).

METHODS: The clinicopathological characteristics of 34 patients with sclerosing cholangitis (SC) associated with AIP were compared with those of 4 patients with PSC.

RESULTS: SC with AIP occurred predominantly in elderly men. Obstructive jaundice was the most frequent initial symptom in SC with AIP. Only SC patients with AIP had elevated serum IgG4 levels, and sclerosing diseases were more frequent in these patients. SC patients with AIP responded well to steroid therapy. Segmental stenosis of the lower bile duct was observed only in SC patients with AIP, but a beaded and pruned-tree appearance was detected only in PSC patients. Dense infiltration of IgG4-positive plasma cells was detected in the bile duct wall and the periportal area, as well as in the pancreas, of SC patients with AIP.

CONCLUSION: SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.

Keywords: Autoimmune pancreatitis, IgG4, Primary sclerosing cholangitis, Sclerosing cholangitis