Science and medicine

Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is a rare autoimmune liver disease in which the bile ducts in the liver are attacked and destroyed by the body’s own immune system. Various immune cell types have been described to participate in the autoimmune response including macrophages, B and T cells. As the disease progresses, the loss of bile ducts results in a toxic build-up of bile acids in the liver leading to liver damage and eventually the need for liver transplantation. The disease primarily affects middle-aged women. PBC is characterized by abnormal high blood levels of the liver enzyme alkaline phosphatase (ALP). Elevated blood ALP levels are used as the primary means of diagnosis and are closely monitored in patients as an indicator of response to therapy. In recent years, it has become univocally accepted that an inappropriately activated immune response is one of the most important factors in PBC. Inhibiting the CD40 pathway in PBC may dampen the autoreactive immune response and thereby improve liver function and symptoms of the disease.