Epidemiological features of immunoglobulin G4-related sclerosing cholangitis in Japan.

Abstract

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.

• Authors: Atsushi Tanaka, Mitsuru Mori, Kensuke Kubota, Itaru Naitoh, Takahiro Nakazawa, Hajime Takikawa, Michiaki Unno, Terumi Kamisawa, Shigeyuki Kawa, Kazuichi Okazaki
• Journal: Journal of hepato-biliary-pancreatic sciences (J Hepatobiliary Pancreat Sci) Vol. 27 Issue 9 Pg. 598-603 (Sep 2020) ISSN: 1868-6982 [Electronic] Japan
• PMID: 32603554 (Publication Type: Journal Article)
• Copyright: © 2020 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

Chemical References

• Immunoglobulin G

Topics

• Aged
• Aged, 80 and over
• Autoimmune Diseases
• Cholangitis, Sclerosing (diagnosis, epidemiology)
• Diagnosis, Differential
• Female
• Humans
• Immunoglobulin G
• Japan (epidemiology)
• Male
• Middle Aged
• Survival Rate