[Primary sclerosing cholangitis: diagnosis, prognosis and treatment]

Primary sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation with fibrosis and obliteration of the intra- and extrahepatic bile ducts. This disease is usually associated with ulcerative colitis. The process of chronic cholestasis eventually leads to biliary cirrhosis. The prevalence of primary sclerosing cholangitis is low in southern Europe but is especially high in Scandinavian countries. The etiopathogenesis is unknown but immune disorders, potential toxic agents or intestinal infections, ischemic injury to the bile ducts, and possibly alterations in hepatobiliary transporters are known to play a role. The disease manifests at the age of approximately 40 years, mainly in men with clinical and laboratory features of cholestasis but may also be asymptomatic. There are specific forms in which the small intrahepatic bile ducts are involved, mainly affecting children, as well as overlap syndromes with autoimmune hepatitis. A form characterized by an increase in IgG4 has been described, which is usually associated with autoimmune pancreatitis. The key diagnostic procedure is endoscopic retrograde cholangiography, although magnetic resonance cholangiography is the first diagnostic procedure that should be used since it is equally informative and non-invasive. Liver biopsy is not essential for diagnosis. Primary sclerosing cholangitis is a progressive disease with a probability of transplant-free survival of 18 years in asymptomatic forms and of 8.5 years in symptomatic forms. Cholangiocarcinoma can result from the disease and confers a poor prognosis. There is no specific treatment although ursodeoxycholic acid improves the biochemical alterations of cholestasis. Liver transplantation is the last therapeutic resort with good results in terms of survival although the disease can recur in the transplanted liver.