Takikawa H, Manabe T
Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
J Gastroenterol. 1997 Feb;32(1):134-7. doi: 10.1007/BF01213311.
Primary sclerosing cholangitis is very rare in Japan. The aim of the present study was to identify the characteristics of such patients in Japan. A questionnaire was sent to the members of the Japanese Society of Gastroenterology and responses for 192 cases were analyzed. There was male predominancy (61%), and two peaks in the age distribution at diagnosis (20-30 years and 50-70 years). Bile duct damage was mainly intra + extrahepatic (69%) versus intrahepatic (17%) or extrahepatic (14%). The incidences of eosinophilia and positivity for anti-nuclear antibody were 27% and 30%, respectively. The incidence of associated inflammatory bowel disease was 21% (38 ulcerative colitis and 2 Crohn's disease). Chronic pancreatitis, gallstones, and biliary cancers occurred in 15%, 12%, and 4%, respectively, of the 192 patients. Patients less than 40 years of age had a higher incidence than the patients 40 years old or more of damage intra + extrahepatic bile ducts (89% vs 56%) and of associated ulcerative colitis (36% vs 9%), whereas the incidence of chronic pancreatitis was lower in patients aged less than 40 years (4%). The characteristics of patients with primary sclerosing cholangitis in Japan differ from those in other countries in regard to age distribution and the incidence of complications, and at least two different groups of patients seem to exist in terms of the degree of bile duct damage and the incidence of complications.
原发性硬化性胆管炎在日本非常罕见。本研究的目的是确定日本此类患者的特征。向日本胃肠病学会成员发送了一份调查问卷，并对192例患者的回复进行了分析。男性占主导（61%），诊断时年龄分布有两个高峰（20 - 30岁和50 - 70岁）。胆管损伤主要为肝内 + 肝外（69%），而肝内（17%）或肝外（14%）损伤较少。嗜酸性粒细胞增多症和抗核抗体阳性的发生率分别为27%和30%。合并炎症性肠病的发生率为21%（38例溃疡性结肠炎和2例克罗恩病）。192例患者中，慢性胰腺炎、胆结石和胆管癌的发生率分别为15%、12%和4%。年龄小于40岁的患者肝内 + 肝外胆管损伤（89%对56%）和合并溃疡性结肠炎（36%对9%）的发生率高于40岁及以上患者，而年龄小于40岁的患者慢性胰腺炎的发生率较低（4%）。日本原发性硬化性胆管炎患者在年龄分布和并发症发生率方面与其他国家不同，并且在胆管损伤程度和并发症发生率方面似乎至少存在两组不同的患者。
