Primary biliary cirrhosis (PBC) is a long-term liver disease in which the bile ducts in the liver become damaged. This gradually leads to a build-up of bile in the liver, which can damage it and eventually lead to scarring (cirrhosis). In PBC, for reasons not fully understood, the immune system (the body's natural defence against infection and illness) mistakenly attacks the bile ducts.
PBC is a relatively rare disease, with some studies showing that it affects up to 1 in 3–4,000 people. The sex ratio is at least 9:1 female to male. As cirrhosis is only a feature of advanced disease, a change of name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014. Use of the new name was advocated in the medical literature in September 2015.
Ursodeoxycholic acid (UDCA) is the major medication used to slow the progression of the disease. Methotrexate may produce improvement in biochemical and histologic findings Corticosteroids may alleviate symptoms and improve biochemical and histologic findings Cyclosporine has some therapeutic potential Colchicine has been used with limited effect