Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Hokkaido Chitose College of Rehabilitation, Chitose, Japan.
J Hepatobiliary Pancreat Sci. 2020 Sep;27(9):598-603. doi: 10.1002/jhbp.793. Epub 2020 Aug 15.
BACKGROUND/PURPOSE: Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce.
We randomly selected healthcare facilities from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with IgG4-SC in 2018. Among responding facilities, we further inquired about the clinical characteristics of the patients with IgG4-SC.
We selected 1180 departments from health centers across Japan. Of them, 532 (45.1%) responded to the questionnaires. The number of reported patients with IgG4-SC was 1045, and the estimated point prevalence was 2.18 (95% confidence interval, 2.13-2.23) per 100 000 population. In addition, we enrolled 1096 patients with IgG4-SC, combining data from previously and newly registered patients. Male patients comprised 79.5% of the population and patients aged 60-80 years had the highest risk for developing the disease. The follow-up period was 5.1 ± 4.0 years and the 5- and 10-year overall survival rates were 95.3% and 89.0%, respectively.
The point prevalence of IgG4-SC was 2.18 per 100 000 population. The epidemiological features were similar to those observed in previous studies.
背景/目的：关于免疫球蛋白 G4 相关硬化性胆管炎（IgG4-SC）的流行病学数据很少。
我们从日本所有医疗机构的名单中随机选择医疗机构。根据基于设施特征和规模的分层确定选择率。我们向选定的医疗机构发送问卷，询问 2018 年 IgG4-SC 患者的数量和性别。在做出回应的医疗机构中，我们进一步询问了 IgG4-SC 患者的临床特征。
我们从全日本的健康中心中选择了 1180 个科室。其中，有 532 个（45.1%）科室回应了问卷调查。报告的 IgG4-SC 患者人数为 1045 人，估计点患病率为每 10 万人 2.18（95%置信区间，2.13-2.23）。此外，我们结合了以前和新登记的患者数据，共纳入了 1096 名 IgG4-SC 患者。男性患者占总人数的 79.5%，60-80 岁的患者发病风险最高。随访时间为 5.1±4.0 年，5 年和 10 年的总生存率分别为 95.3%和 89.0%。
IgG4-SC 的点患病率为每 10 万人 2.18 人。流行病学特征与之前的研究观察结果相似。
