Primary biliary cirrhosis: historical perspective

PBC is an old disease first described in 1851. It's predilection for women and its association with other autoimmune diseases suggests an immune based pathogenesis, but epidemiologic studies indicate that genetic and environmental factors play a role in the pathogenesis of PBC. The serologic hallmark for PBC, namely the antimitochondrial antibody, was first identified in 1965 and remains the most sensitive and specific hallmark for this disease. When first described, primary biliary cirrhosis was universally fatal but asymptomatic cases now represent more than 60% of cases diagnosed, less than half of whom will die of their liver disease. No specific therapy that effectively stops or reverses disease progression has been identified, thus it behoves investigators to aggressively pursue identification of the etiology of PBC.