Primary biliary cirrhosis
Primary biliary cirrhosis is a predominantly middle-aged female autoimmune liver disease (F:M = 9:1), which is associated with other autoimmune diseases. It is a disease of unknown cause that is characterized by chronic cholestasis and by progressive destruction of intrahepatic bile ducts of the liver. The presence of granulomas suggests a delayed-type hypersensitivity reaction. As the disease progresses, the small ducts are destroyed, and cirrhosis develops due to cholestasis. Granulomas are present in 30-70% of cases.
Individuals have minimal symptoms such as fatigue and occasionally pruritus. The anti-mitrochondrial antibody (AMA) is present in over 95% of individuals with PBC and has extremely high sensitivity and specificity for this disease. A liver biopsy can also confirm the diagnosis. The main medical treatment to date is the use of Ursofalk. Many immunosuppressive agents have been used but really to no benefit and Ursofalk has clearly been shown to reduce the incidence of liver-related deaths and the need for liver transplantation.