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© 2017 Elsevier B.V. Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease caused by diffuse inflammation and concentric obliterative fibrosis that can involve the entire biliary tree. It is usually a progressive disorder that can ultimately lead to biliary cirrhosis, portal hypertension, and hepatic failure. PSC is a complex genetic disease: environmental predisposing factors include nonsmoking. Although the underlying etiopathogenesis of PSC is not yet fully elucidated, it is generally accepted to be a condition of immune dysregulation. Unlike most immune-mediated conditions, PSC tends to affect men (male:female—2:1). While PSC is increasingly recognized as a heterogeneous group of disorders, the majority of cases are closely associated with inflammatory bowel disease (IBD), particularly ulcerative colitis, which occurs in about two-thirds of PSC cases. Recent studies have suggested that PSC/IBD is a separate disease entity from IBD alone with distinctive genetic and phenotypic characteristics. Most PSC/IBD patients are asymptomatic at presentation, although clinical symptoms include fatigue, jaundice, weight loss, right upper quadrant pain, and pruritus. Serum biochemical tests indicate cholestasis, and diagnosis is usually established by cholangiography. In symptomatic patients, median survival from presentation to death or liver transplantation is about 12 years, although the overall median survival is 21 years. It is a premalignant condition, and the majority of deaths are from malignancy, particularly cholangiocarcinoma or colonic cancer. PSC has no curative treatment. Medical treatment with ursodeoxycholic acid may slow progression of the disease, although clinical trials lack statistical significance. Liver transplantation is the only option in young patients with PSC and advanced liver disease.

Original publication

DOI

10.1016/B978-0-444-63707-9.00007-6

Type

Chapter

Book title

Handbook of Systemic Autoimmune Diseases

Publication Date

01/01/2017

Volume

13

Pages

119 - 139