title: Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis.
abstract: OBJECTIVE
  We encountered one group of primary sclerosing cholangitis cases which met classic criteria, but demonstrated better clinical courses.
  
  
  METHODS
  We evaluated the clinical characteristics of several cases of primary sclerosing cholangitis, especially those associated with chronic pancreatitis.
  
  
  RESULTS
  Of a total of 25 cases, eight had a background of pancreatitis with unusual irregularly segmental or diffuse narrowing features on pancreatography. Abdominal ultrasonography and computed tomography also revealed segmental or diffuse enlargement of the pancreas. Pathological findings of surgical specimens in two cases pointed to lymphoplasmacytic sclerosing pancreatitis. The walls of the lower biliary ducts were also thickened with a similar appearance to the pancreatic ducts. In seven cases, cholangiography displayed stenosis of the lower part of the common bile duct and in one case, only the hepatic hilar region was affected. In this case both biliary and pancreatic lesions responded to steroid therapy simultaneously. One case developed hepatic hilar stenosis four months after a lesion in the lower part of the common bile duct had disappeared. The cases of primary sclerosing cholangitis with pancreatitis demonstrated better clinical courses than did typical cases of primary sclerosing cholangitis. Six cases were characterized by autoimmunological abnormalities.
  
  
  CONCLUSIONS
  We concluded that similar etiological agents might impact on both the pancreas and biliary tract, either simultaneously or in sequence. We propose new criteria for these atypical primary sclerosing cholangitis cases.
authors.affiliation: First Department of Internal Medicine, Nagoya City University Medical School, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467-8601, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Nakazawa
authors.forename: H
authors.initials: H
authors.lastname: Ohara
authors.forename: T
authors.initials: T
authors.lastname: Yamada
authors.forename: H
authors.initials: H
authors.lastname: Ando
authors.forename: H
authors.initials: H
authors.lastname: Sano
authors.forename: S
authors.initials: S
authors.lastname: Kajino
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: S
authors.initials: S
authors.lastname: Nakamura
authors.forename: T
authors.initials: T
authors.lastname: Ando
authors.forename: T
authors.initials: T
authors.lastname: Nomura
authors.forename: T
authors.initials: T
authors.lastname: Joh
authors.forename: M
authors.initials: M
authors.lastname: Itoh
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000473:pathology; D017734:Bile Ducts, Extrahepatic / Q000473:pathology; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000473:pathology; D002908:Chronic Disease; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D005260:Female; D006801:Humans; D008111:Liver Function Tests; D008297:Male; D008875:Middle Aged; D010179:Pancreas / Q000473:pathology; D010183:Pancreatic Ducts / Q000473:pathology; D010195:Pancreatitis / Q000175:diagnosis* / Q000473:pathology