Clinical Gastroenterology Vol.29 No.11(2-1)

Theme PSC and PBC -- Recent Progress in Diagnosis and Treatment
Title Epidemiology and Prognosis of Primary Biliary Cirrhosis in Japan
Publish Date 2014/10
Author Junko Hirohara Third Department of Internal Medicine, Kansai Medical University
Author Toshiaki Nakano University Information Center, Kansai Medical University
Author Toshihito Seki Third Department of Internal Medicine, Kansai Medical University
Author Kazuichi Okazaki Third Department of Internal Medicine, Kansai Medical University
Author Yasuni Nakanuma Shizuoka Cancer Center
Author Hirohito Tsubouchi Kagoshima City Hospital
[ Summary ] Multicenter national surveys of primary biliary cirrhosis (PBC) have been carried out 15 times since 1980 in Japan by members of the Intractable Hepatobiliary Disease Study Group. These surveys have been supported by a Health Labour Sciences Research Grant. We investigated the epidemiological trends and long-term prognose for PBC patients in Japan, based on national surveys. The annual incidence expressed as the number of PBC patients nearly leveled off after 1989. Approximately 70 % of patients with PBC were diagnosed in the asymptomatic stage. The prevalence of PBC gradually increased throughout the observation period. PBC predominantly affects middle-aged women, with a female/male ratio of 7:1. Recently, the annual number of deaths caused by liver disease has decreased. As a result of examining changes in survival rates for PBC patients at individual clinical stages, a significant difference was observed between the rates in the 1980s, 1990s and 2000s for of a-PBC and s-PBC. From the study of long-term prognoses for PBC in Japan based on national surveys, the prognosis for patients in all clinical stages of PBC has improved.
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