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Table 1A. International Union of Immunological Societies
(IUIS) Classification of Human IEI 2024
Subtable VI Defects in intrinsic and innate immunities
1. Mendelian susceptibility to mycobacterial disease (MSMD)
2. Epidermodysplasia verruciformis (human papilloma virus [HPV])
3. Predisposition to severe viral infections
4. Herpes simplex encephalitis
5. Predisposition to invasive fungal disease
6. Predisposition to mucocutaneous candidiasis
7. Toll-like receptor (TLR) signaling pathway deficiency
8. Other IEI related to non-hematopoietic tissues
9. Other IEI related to leukocytes
Subtable VII Autoinflammatory disorders
1. Type I interferonopathies
2. Defects affecting the inflammasome
3. Non-inflammasome–related conditions
Subtable VIII Complement deficiencies
Subtable IX Bone marrow failures
Subtable X Phenocopies of IEI
Table 1B. Examples of Secondary Causes of
Immunodeficiency
Anatomical: nasal polyposis, deviated septum, adenoidal hypertrophy, cerebrospinal
fluid (CSF) leak
Defective epithelial barriers: primary ciliary dyskinesia, cystic fibrosis
Malnutrition
Infections targeting immune cells: human immunodeficiency virus (HIV) infection,
measles, EBV infection
Use of immunosuppressive medication: corticosteroids, cyclosporine, rituximab
Lymphatic system malformations
Prematurity and advanced age
(cont'd)
