Steroid-Responsive (Autoimmune?) Sclerosing Cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a chronic, progressive cholestatic liver disease characterized by inflammation, fibrosis and destruction of the intrahepatic and extrahepatic bile ducts (1). Although the natural history of PSC is variable, it is almost always progressive and leads to cirrhosis and liver failure (2‐5). The etiology of PSC is unknown, and there is no effective medical treatment. We report on all patients who first presented to us between January 1999 and December 2001 with confirmed sclerosing cholangitis of a highly inflammatory nature. Six such patients presented in this time-period out of 80 patients with PSC. All six of these patients improved dramatically in response to glucocorticoid treatment. Biochemical tests of liver function normalized in five patients and improved greatly in the sixth, cholangiograms improved in those who had cholangiograms before and after treatment and symptoms resolved in all six. The response of these patients to immunosuppressive therapy suggests that their sclerosing cholangitis may be of autoimmune etiology. Based on this experience, we suggest that PSC may be a syndrome with different etiologies, rather than one discrete disease, and that our patients have an autoimmune variant. That sclerosing cholangitis may be a family of diseases with different etiologies would explain the disparate results found in the PSC literature concerning the efficacy of medical treatments.