Primary biliary cholangitis: A historical perspective from xanthomatous lesions to modern molecular biology.

Abstract

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by damage and loss of the epithelial lining of small intrahepatic bile ducts, leading to ductopenia and cholestasis. In advanced stages, this process results in cirrhosis and liver failure. The disease belongs to cholangiopathies. The review addressed historical questions concerning: The history of the first mention of this disease; how its nomenclature was formed; when specific serological tests were discovered and their importance in the diagnosis of PBC; the history of ursodeoxycholic and other bile acids for the treatment of PBC; and the significance of modern data on impaired bicarbonate production by cholangiocytes in the pathogenesis of PBC.