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 pro-gresses 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.

Primary biliary cholangitis(PBC)is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts.Although the pathogenesis of PBC is not completely understood,it is charac-terized by cholestatic inflammation resulting in a spectrum of cholangitis,varying degrees of fibrosis,and,potentially,cirrhosis.PBC affects all races;however,there have been few clinical studies of the disease in Asian countries,particularly in Southeast Asia,suggesting that PBC may be underreported in the Asian population.Here,we review the epidemiology,natural history,predictive and diagnostic features,and current and emerging treatments for PBC.Our goal is also to highlight current studies from Asian countries to provide a better understanding of PBC in this patient population.

Cholestatic liver diseases(CLDs)encompass a variety of disorders of abnormal bile formation and/or flow.CLDs often lead to progressive hepatic insult and injury and following the development of cirrhosis and associated complications.Many such complications are clinically silent until they manifest with severe sequelae,including but not limited to life-altering symptoms,metabolic disturbances,cirrhosis,and hepatobiliary diseases as well as other malignancies.Primary sclerosing cholangitis(PSC)and primary biliary cholangitis(PBC)are the most common CLDs,and both relate to mutual as well as unique complications.This review provides an overview of PSC and PBC,with a focus on preventive measures aimed to reduce the incidence and severity of disease-related complications.