Benign recurrent intrahepatic cholestasis: a long-term follow-up

We report on a case with a 50-year history of benign recurrent intrahepatic cholestasis, including 33 years of follow-up at our hospital. The attacks of jaundice began at the age of 1 year and have been repeated more than 14 times to date. During the follow-up period at our hospital, the patient has suffered 6 attacks of cholestasis. During the episodes of cholestasis, a dissociation between the serum level of bilirubin and that of bile acids was observed. The analysis of serum bile acids during cholestasis showed that cholic acid and chenodeoxycholic acid were the main bile acids, and no appreciable amounts of the secondary bile acids were detected. Between episodes the liver function tests, including serum bile acids, were normal. Despite multiple attacks of cholestasis over many years, no permanent liver damage has occurred. This case reaffirms the essentially benign nature of this disease.