期刊
JOURNAL OF HEPATO-BILIARY-PANCREATIC SCIENCES
卷 27, 期 9, 页码 598-603出版社
WILEY
DOI: 10.1002/jhbp.793
关键词
autoimmune pancreatitis; epidemiology; etiology; incidence; prevalence
资金
- Intractable Hepatobiliary Disease Study Group in Japan - Ministry of Health, Labor, and Welfare of Japan
- Research Committee to establish diagnostic criteria and development of treatment for systemic IgG4-related sclerosing disease - Ministry of Health, Labor, and Welfare of Japan
Background/Purpose Epidemiological data on immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) are scarce. Method 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. Results 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. Conclusions The point prevalence of IgG4-SC was 2.18 per 100 000 population. The epidemiological features were similar to those observed in previous studies.
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