臨牀消化器内科 Vol.29 No.11(2-1)


特集名 PSCとPBC ― 診断と治療の進歩
題名 原発性胆汁性肝硬変(PBC)(1) PBCの疫学と予後
発刊年月 2014年 10月
著者 廣原 淳子 関西医科大学内科学第三講座
著者 仲野 俊成 関西医科大学大学情報センター医療情報部
著者 関 寿人 関西医科大学内科学第三講座
著者 岡崎 和一 関西医科大学内科学第三講座
著者 中沼 安二 静岡県立静岡がんセンター病理診断科
著者 坪内 博仁 鹿児島市立病院
【 要旨 】 厚生労働科学研究費補助金難治性疾患克服研究事業「難治性の肝・胆道疾患に関する調査研究」班では約30年間にわたり,原発性胆汁性肝硬変(PBC)全国調査を継続実施してきた.全国調査登録症例の解析では,新規発生数は近年横ばいであるが,有病者数は年々増加し,新たに診断される症例のうち約70%以上は無症候性PBCである.男女比は1:7,最頻年齢は5〜60歳代で,診断時年齢は年々高齢化傾向にある.おもな死因は肝不全と消化管出血であるが肝疾患関連死亡数は減少傾向にあり,無症候性,症候性PBCとも年代別生存率解析では有意に生命予後は改善している.
Theme PSC and PBC -- Recent Progress in Diagnosis and Treatment
Title Epidemiology and Prognosis of Primary Biliary Cirrhosis in Japan
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.
戻る