Changing Nomenclature for PBC from “Primary Biliary Cirrhosis” to “Primary Biliary Cholangitis”

Excerpt

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, frequently developing in middle-aged women [1]. In patients with PBC, small bile ducts in the liver are believed to be damaged via autoimmune reactions [2]. Patients were diagnosed as having PBC when 2 or more among the following 3 features are observed; elevated cholestatic enzymes, presence of anti-mitochondrial autoantibodies (AMA) and histological findings as chronic non-suppurative destructive cholangitis (CNSDC) in particular [3]. When the disease entity of PBC was established more than 50 years ago [4], advanced liver diseases showing histological findings of cirrhosis were found in the most of patients with PBC. Consequently, a nomenclature for primary biliary “cirrhosis” was reasonably accepted by all hepatologists and gastroenterologists world-widely. …