title: Sclerosing cholangitis associated with autoimmune pancreatitis differs from primary sclerosing cholangitis.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  SC with AIP is distinctly different from PSC. The two diseases can be discriminated based on cholangiopancreatographic findings and serum IgG4 levels.
authors.affiliation: Department of Internal Medicine, Tokyo Metropolitan Komagome Hospital, 3-18-22 Honkomagome, Bunkyo-ku, Tokyo, Japan. kamisawa@cick.jp
authors.forename: Terumi
authors.initials: T
authors.lastname: Kamisawa
authors.forename: Kensuke
authors.initials: K
authors.lastname: Takuma
authors.forename: Hajime
authors.initials: H
authors.lastname: Anjiki
authors.forename: Naoto
authors.initials: N
authors.lastname: Egawa
authors.forename: Masanao
authors.initials: M
authors.lastname: Kurata
authors.forename: Goro
authors.initials: G
authors.lastname: Honda
authors.forename: Kouji
authors.initials: K
authors.lastname: Tsuruta
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications*; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007150:Immunohistochemistry; D008297:Male; D010195:Pancreatitis / Q000150:complications*
chemical_list: D007074:Immunoglobulin G