Primary Biliary Cholangitis

Primary biliary cholangitis, previously called primary biliary cirrhosis, is an autoimmune disease of the liver in which the small bile ducts are progressively destroyed, letting bile and toxins build up in a state called cholestasis that can eventually cause scarring, fibrosis, and cirrhosis. Most patients carry antimitochondrial antibodies directed against a specific mitochondrial enzyme complex, and genetic predisposition plays a real role, shown by clustering within families and genome-wide studies linking multiple immune-regulation genes to the disease. Tiredness affects about 80 percent of patients and itching occurs in 20 to 70 percent, with jaundice appearing in advanced disease, though in early cases the only sign may be abnormal blood tests. It affects roughly 1 in 3,000 to 4,000 people, at least nine times more often in women than men, and was formally renamed in 2014 because cirrhosis is only a late feature of the disease rather than something present from the start.