Revisão narrativa sobre colangite esclerosante primária

Abstract

Primary sclerosing cholangitis is a chronic, cholestatic liver disease that can involve the entire biliary tree caused by diffuse inflammation, fibrosis and stenosis. The pathogenesis is unkown, but it is established as being imune mediated. It is a progressive disorder which can ultimately lead to biliary cirrhosis, portal hypertension and hepatic failure. Most patients are asymptomatic at presentation.The diagnosis is established by cholangiography and serum biochemical tests indicate cholestasis. Liver biopsy is not routinely indicated. There is a higher risck of malignacy, above all, cholangiocarcinoma, gallbladder and colorectal cancer. Primary sclerosing cholangitis has no curative treatment,except liver transplant. Medical treatment with ursodeoxycholic acid may improve laboratory findings. There is no medical treatment capable of slow progression of the disease and outcome. This is a narative review about the main topics related to primary sclerosing cholangitis, which includes, epidemiology, pathogenesis, clinical features, diagnosis, prognosis malignacy screening and treatment.