Curriculum · Gastroenterology
Primary biliary cholangitis
What it is
Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic progressive autoimmune cholestatic liver disease. It is a disease of chronic cholestasis, meaning decreased bile acid flow, and it is a disease of the small bile ducts. It occurs predominantly in women aged 40 to 60 years.
Causes and risk
The disease is autoimmune, and it is associated with other autoimmune diseases such as Sjogren syndrome, rheumatoid arthritis, Hashimoto thyroiditis and scleroderma. It is also associated with Sicca syndrome of dry eyes and dry mouth, with Raynaud's phenomenon, and with CREST syndrome, the limited form of systemic sclerosis. This group of autoimmune disease is one of the things that separates it from primary sclerosing cholangitis, which is associated with IBD instead.
How it presents
Characteristic findings are pruritus, fatigue, weight loss, hyperpigmentation, and complications of portal hypertension. Approximately 50% of patients are asymptomatic.