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NEW! - Pathogenesis of primary biliary cholangitis (primary biliary cirrhosis)

Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack, primarily on small interlobular bile ducts, but can also involve the smaller intralobular bile ducts, ie, bile canaliculi. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis, and eventually results in cirrhosis and liver failure.

Clinical manifestations, diagnosis, and prognosis of primary biliary cholangitis

Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is characterized by a T-lymphocyte-mediated attack on small intralobular bile ducts. A continuous assault on the bile duct epithelial cells leads to their gradual destruction and eventual disappearance. The sustained loss of intralobular bile ducts causes the signs and symptoms of cholestasis and eventually may result in cirrhosis and liver failure. The terminology was changed from primary biliary cirrhosis to primary biliary cholangitis to more accurately describe the disorder and its natural history. With the advent of treatment with ursodeoxycholic acid, the majority of patients have normal life expectancies and only a minority of patients develop cirrhosis.