Primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) remains a disease of unknown etiology. The close association of PSC and inflammatory bowel disease (IBD), especially ulcerative colitis (UC), has been reconfirmed in numerous studies. Much has been learned about the pathogenesis, although the specific cause remains unknown. Copper overload and chronic hepatic bacterial infection have virtually been excluded as causes of PSC. Cytomegalovirus and reovirus remain under investigation. Familial clustering and HLA subtype similarities are seen in PSC with and without IBD. The finding of antineutrophil cytoplasmic antibodies (ANCA) in patients with PSC and those with UC suggests immunological features in the pathogenesis of PSC. Collected series of patients have better characterized clinical features of PSC. Endoscopic retrograde cholangiopancreatography (ERCP) and percutaneous transhepatic cholangiography (PTC) have provided both diagnostic features and means of therapeutic intervention. Treatment of PSC is symptomatic (pruritus control and vitamin deficiency correction); or experimental (D-penicillamine, ursodeoxycholic acid [UDCA], methotrexate, or corticosteroids). Liver transplantation remains the ultimate treatment for end-stage PSC. Statistical analyses of clinical and laboratory variables in PSC help to determine prognosis and proposed timing for transplantation to achieve maximal longevity and quality of life. PSC affects middle-aged people and is expensive to treat over the natural course of the disease, making it an economically and medically important disease.