IUIS classification of IEI diseases

IUIS classificationPID disease categoryDisorders# of diseases# of genetic defects
1Cellular and hormonal immunodeficienciesSCID5866
2Syndromic combined immunodeficienciesT cells and B cells6869
3Predominantly antibody deficienciesHypogammaglobulinemia5145
4Immune dysregulation diseaseHemophagocytic lymphohistiocytosis5152
5Congenital defects of phagocytesDefects in phagocyte function3542
6Defects in intrinsic and innate immunityBacterial, fungal, and viral infections6374
7Autoinflammatory diseasesAutoinflammatory diseases5956
8Complement deficienciesDecrease in the levels of a component protein3036
9Bone marrow failure diseasesReduction in the levels of red and white blood cells4344
10Phenocopies of inborn errors of immunityVarious diseases1515*

* It was assumed that these are genetic defects or pathogenesis