Growth in diagnosis and treatment of primary immunodeficiency within the global Jeffrey Modell Centers Network

Table 2 Physician-reported prevalence of PI by classification, 2021

	US	INT’L	Global
Immunodeficiencies affecting cellular and humoral immunity	1566	5985	7551
Combined immunodeficiencies with associated syndromic features	5677	10,892	16,569
Predominantly antibody deficiencies	19,423	37,418	56,841
Diseases of immune dysregulation	1199	3676	4875
Congenital defects of phagocyte number or function	1678	17,332	19,010
Defects in intrinsic and innate immunity	311	1993	2304
Autoinflammatory disorders	673	6328	7001
Complement deficiencies	646	4418	5064
Bone marrow failure	58	306	364
Phenocopies of inborn errors of immunity	48	286	334
Unspecified or other deficiencies	3135	12,362	15,497
Total^a	34,414	100,996	135,410

The 2021 distribution of patients according to the IUIS classification of PI categories
^aGlobal total doesn’t match Table 1 due to the inclusion of “Unspecified or Other Deficiencies” and reporting

ISSN: 1710-1492