What we once called "primary biliary cirrhosis" is now termed "primary biliary cholangitis" (PBC).[1] The original name never quite fit, given that patients with PBC usually do not have cirrhosis at the time of clinical presentation.[2] "Chronic nonsuppurative destructive cholangitis" was an early attempt at renaming the disorder,[3] but "primary biliary cirrhosis" was too ingrained in the language of physicians at that time, so it never caught on.
PBC is a cholestatic, autoimmune disorder characterized by intrahepatic bile duct destruction, mononuclear portal inflammation, cholestasis, periportal fibrosis, and eventual cirrhosis.[4] The disorder predominantly affects women (nine women for every man) and usually manifests in midlife between 30 and 65 years of age, though it can develop earlier or later in age as well.[4] The majority of patients (95%) will have circulating antimitochondrial antibodies (AMAs) that develop before the onset of clinical disease.[5] AMAs are also observed in approximately 1% of otherwise normal adults.
Once a diagnosis of PBC is established, progression to cirrhosis within 4 years can occur in untreated patients.[2]Today, patients are often diagnosed at earlier stages, so the time of progression to advanced disease may take longer. Progression of disease is also delayed by currently available effective therapies.
