Martins EB., Chapman RW.
Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown etiology that causes progressive obliterative fibrosis of the biliary tree, ultimately leading to death from liver failure or cholangiocarcinoma. The majority of patients have a circulating antineutrophil cytoplasmic antibody, which does not have a high enough sensitivity to be a good disease marker. Patients with ulcerative colitis and sclerosing cholangitis are at a higher risk for the development of colonic dysplasia and neoplasia, and yearly colonoscopic surveillance may be warranted. To date there is no effective treatment. Endoscopic stenting may be tried in selected cases. Orthotopic liver transplantation remains the only proven therapeutic option in end stage disease. Five-year survival after liver transplantation is 85%. However, if a cholangiocarcinoma is present at the time of transplantation survival decreases markedly. The disease may recur after transplantation, but this does not appear to have any clinical implications. Post-tranplant patients who have ulcerative colitis who are receiving immunossuppresive therapy are also at a high risk for the development of colon cancer.