Primary sclerosing cholangitis: A review and update

Abstract

Primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease of uncertain etiology characterized biochemically by cholestasis and histologically and cholangiographically by fibro-obliterative inflammation of the bile ducts. In a clinically significant proportion of patients, PSC progresses to cirrhosis, end-stage liver disease, and/or hepatobiliary cancer, though the disease course can be highly variable. Despite clinical trials of numerous pharmacotherapies over several decades, safe and effective medical therapy remains to be established. Liver transplantation is an option for select patients with severe complications of PSC, and its outcomes are generally favorable. Periodic surveillance testing for pre-as well as post-transplant patients is a cornerstone of preventive care and health maintenance. Here we provide an overview of PSC, including its epidemiology, etiopathogenesis, clinical features, associated disorders, surveillance, and emerging potential therapies. Keywords: Bile duct diseases, Primary sclerosing cholangitis (PSC), Cholestasis, Cholangiocarcinoma, Magnetic resonance imaging, Cholangiography, Natural history