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. 2022 Jun 24;42(7):1473–1507. doi: 10.1007/s10875-022-01289-3

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© The Author(s) 2022

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 1 — Accumulative discovery of novel inborn errors of immunity: 1980–2022. (A) The number of genetic defects underlying monogenic immune disorders as reported in the indicated year. (B) The number of pathogenic variants listed in each Table of the IUIS IEI committee 2022 report. The numbers in each column correspond to the number of genes reported in the 2019 IUIS update (blue bars) [4, 5], the number of new genes for each Table contained in this report (red bars), and the total number of genes for each Table (black number). Note: The 14 conditions listed for Table 10 are either phenocopies of germline IEI due to somatic variants or neutralizing autoAbs. Somatic variants in UBA1 are also listed in Table 10, although there is currently no IEI resulting from germline UBA1 variants [97]