[PRIMARY SCLEROSING CHOLANGITIS]

Primary sclerosing cholangitis (PSC) is a chronic cholestatic and progressive disease, of unknown etiology, characterized by inflammation and fibrosis of intra-hepatic and extra-hepatic biliary tree. Two thirds of the patients have, simultaneously, ulcerative colitis (UC); on the other hand, PSC is the most common chronic hepatic disease in patients with inflammatory bowel disease (IBD). Patients who have both diseases simultaneously are prone to develop colorectal carcinoma and cholangiocarcinoma. The disease predominantly affects young men, may be asymptomatic or presented as fluctuating jaundice, pruritus and increased levels of the cholestasis biochemical markers, or as secondary billiary cirrhosis. The diagnostic gold standard are the cholangiographic abnormalities, consisting of multifocal stenosis and dilatations, involving both the intrahepatic and the extrahepatic biliary tree. Liver biopsy is useful only for staging the disease and to confirm the diagnosis, in the atypical forms. Ursodeoxycholic acid is a promising drug, nowadays, mainly in the first stages of the disease, in spite of doubtful efficacy. Combination therapy, using ursodeoxicolic acid, methotrexate and alternating antibiotics monthly may be sucessfull in PSC before bile strictures occur. Liver transplantation is the only life-saving therapeutic alternative, able to improve significantly the survival and the life quality of the patients.

PMID: 12140588 [PubMed - as supplied by publisher]