Sclerosing cholangitis in cystic fibrosis

Four of 102 patients with cystic fibrosis with symptoms or signs suggesting sclerosing cholangitis had typical findings at endoscopic retrograde cholangiography (ERC), indicating this syndrome. All patients had pulmonary symptoms, pancreatic insufficiency, and pathologic sweat test results. Three females (aged 15-20 years), two of whom had unspecific colitis, presented with abdominal pain, and a 25-year-old male patient was asymptomatic. Two of the patients had persistently and one patient only intermittently pathologic serum concentrations of transaminases and gamma-glutamyltransferase. The fourth patient, who only had a transient increase of standard liver function tests, showed no progress in any variable (including liver biopsy and ERC) for 3 years, indicating a benign course. Disturbances of the liver and biliary system are well-known complications in cystic fibrosis. Our observations indicate that sclerosing cholangitis is another expression of biliary tract involvement in this disease.