Atypical primary sclerosing cholangitis cases associated with unusual pancreatitis

We encountered one group of primary sclerosing cholangitis cases which met classic criteria, but demonstrated better clinical courses.

We evaluated the clinical characteristics of several cases of primary sclerosing cholangitis, especially those associated with chronic pancreatitis.

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.

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.