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. 2022 Jan;42(1):183-194.
doi: 10.1007/s10875-021-01160-x. Epub 2021 Oct 27.

Vaccination for Patients with Inborn Errors of Immunity: a Nationwide Survey in Japan

Sho Hosaka  1 , Takahiro Kido  2 , Kazuo Imagawa  2   3 , Hiroko Fukushima  2   3 , Tomohiro Morio  4 , Shigeaki Nonoyama  5 , Hidetoshi Takada  2   3
Affiliations

Vaccination for Patients with Inborn Errors of Immunity: a Nationwide Survey in Japan

Sho Hosaka et al. J Clin Immunol. 2022 Jan.

Erratum in

Abstract

We conducted a nationwide survey of inborn errors of immunity (IEI) in Japan for the second time in 10 years, focusing on protective measures for IEI patients against infectious diseases. Questionnaires were sent to various medical departments nationwide, and a total of 1307 patients were reported. The prevalence of IEI was 2.2 patients per 100,000 population, which was comparable with the previous nationwide study. The most common disease category was autoinflammatory disorders (25%), followed by antibody deficiencies (24%) and congenital defects of phagocyte number or function (16%). We found that a significant number of patients received contraindicated vaccines, principally because the patients were not diagnosed with IEI by the time of the vaccination. Regarding diseases for which BCG vaccination is contraindicated, 43% of patients had actually received BCG, of which 14% developed BCG-related infections. BCG-related infections were mainly observed among patients with CGD and MSMD. In order to prevent IEI patients from receiving inadequate vaccines, continuous education to parents and physicians is needed, along with the expansion of newborn screening, but efforts to screen IEI at the site of vaccination also remain important.

Keywords: BCG; Epidemiology; Inborn errors of immunity; Japan; Vaccine.

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