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Epidemiological features of immunoglobulin G4‐related sclerosing cholangitis in Japan
Author(s) -
Tanaka Atsushi,
Mori Mitsuru,
Kubota Kensuke,
Naitoh Itaru,
Nakazawa Takahiro,
Takikawa Hajime,
Unno Michiaki,
Kamisawa Terumi,
Kawa Shigeyuki,
Okazaki Kazuichi
Publication year - 2020
Publication title -
journal of hepato‐biliary‐pancreatic sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.63
H-Index - 60
eISSN - 1868-6982
pISSN - 1868-6974
DOI - 10.1002/jhbp.793
Subject(s) - medicine , epidemiology , confidence interval , population , primary sclerosing cholangitis , pediatrics , disease , environmental health
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.