Primary biliary cirrhosis in Japan: national survey by the Subcommittee on Autoimmune hepatitis.

A total of 280 cases of primary biliary cirrhosis were reported from 86 institutes in Japan, of whom 208 were middle aged women. Four clinical stages (asymptomatic, pruritus, icteric and terminal stage) were set up for analysing clinical and histopathological features based on the natural course of the disease. The clinical and histopathological findings were similar to the study reported from the United States and European countries. Out of 270 cases examined, 245 (90.7%), had mitochondrial antibodies. Concerning the prognosis 37 of 120 asymptomatic patients developed symptoms and the average symptom-free period in these patients was 28.7 months. Fifty-eight cases were fatal and causes of death were hepatic failure in 27, gastrointestinal bleeding in 20 and others in 11 cases. Patients were subdivided into three groups to elucidate the survival rate in patients with different symptoms. Asymptomatic patients showed almost the same survival rate as the patients with pruritus alone, showing about 50% survival 8 years after the diagnosis, in contrast to jaundiced cases with only 13.6% surviving 8 years after the onset of this symptom.