The 6 C’s of Primary Sclerosing Cholangitis (PSC)
PSC is a chronic, cholestatic, immune-mediated disease characterized by progressive inflammation, fibrosis, and destruction of intra- and extra-hepatic bile ducts, commonly leading to multi-focal biliary strictures and eventual progression to end-stage liver disease.
• COVERT - PSC affects both sexes and occurs at all ages; a majority of patients are male and the median age at onset is 30–40 years. More than two-thirds of patients will have concomitant IBD. Approximately 50% of patients with PSC are asymptomatic at diagnosis.
• CHOLANGITIS - Genetic and environmental factors interact to establish the pathogenesis of PSC.
• CIRRHOSIS - Progression to cirrhosis is most frequent in symptomatic patients with persistently active disease biochemically. Whilst there are many indications for liver transplantation in PSC, it is mostly indicated for decompensated cirrhosis.
• COLITIS - PSC-IBD is phenotypically distinct from IBD without PSC
• CANCER - PSC patients are at increased risk of colorectal and hepato-biliary cancers, including cholangiocarcinoma, hepatocellular carcinoma, and gallbladder cancer
• CURE - Care focuses on symptom control, judicious use of ERCP, and end-stage liver disease management. Therapy with UDCA remains common but evidence for efficacy is challenged by ongoing outcome data. Some advocate a trial of UDCA for all patietns, with cessation if insignificant biochemical response. Other approaches remain subject to clinical trial evaluation.
Dr. Gideon Hirschfield @AutoImmuneLiver
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