Primary sclerosing cholangitis.

Primary sclerosing cholangitis is now being recognized as the underlying cause of liver disease with increasing frequency. This is due to the widespread use of cholangiography especially endoscopic retrograde cholangiopancreatography. Hepatic histology shows a spectrum of changes and is diagnostic in only a minority of patients. Several distinct clinical presentations of primary sclerosing cholangitis are now recognized. While most patients present with a cholestatic illness and acute cholangitis, a proportion simulate chronic active hepatitis, cryptogenic cirrhosis or primary biliary cirrhosis. Some patients are entirely asymptomatic. Primary sclerosing cholangitis is associated with inflammatory bowel disease (predominantly ulcerative colitis) and a variety of fibrosing diseases. Approximately 3 percent of patients with ulcerative colitis develop primary sclerosing cholangitis. The prognosis of primary sclerosing cholangitis is variable; it can no longer be regarded as an invariably progressive cholestatic disease. Patients may remain asymptomatic or enjoy remissions lasting many years. Treatment for the disease remains unsatisfactory despite attempts with numerous medical and surgical measures.