Table 1.
Percentage of live patients receiving immunoglobulin (Ig) replacement therapy within the largest primary immunodeficiency (PID) subgroups in the European Society for Immunodeficiencies (ESID) database.
| PID subcategory | Alive patients | Patients receiving Ig replacement (%) |
|---|---|---|
| Agammaglobulinaemias | 500 | 92·2 |
| Common variable immunodeficiency (CVID) | 1483 | 86·6 |
| Class switch recombination defects (CSR)/hyper-IgM syndromes | 218 | 57·8 |
| X-linked lymphoproliferative syndrome (XLP) | 36 | 50·0 |
| T-B- severe combined immunodeficiency (SCID) | 143 | 46·2 |
| Wiskott–Aldrich syndrome (WAS) | 235 | 43·8 |
| Other unclassified T cell disorders | 99 | 43·4 |
| Human leucocyte antigen class II deficiency | 31 | 41·9 |
| T-B+ severe combined immunodeficiency (SCID) | 155 | 41·3 |
| Chronic mucocutaneous candidiasis (CMC) | 27 | 29·6 |
| CD4 deficiency | 35 | 28·6 |
| Immunodeficiencies of unknown cause | 111 | 27·0 |
| Hypogammaglobulinaemias (without CVID) | 1823 | 24·4 |
| Hyper-IgE syndromes | 114 | 22·8 |
| DNA-breakage disorder | 353 | 22·1 |
| Familial haemophagocytic lymphohistiocytosis syndromes (FLH) | 52 | 17·3 |
| Autoimmune lymphoproliferative syndrome (ALPS) | 78 | 15·4 |
| Leucocyte adhesion deficiency (LAD) | 30 | 6·7 |
| DiGeorge syndrome | 230 | 4·3 |
| Severe congenital neutropenia and Kostmann syndrome | 258 | 3·9 |
| Chronic granulomatous disease (CGD) | 300 | 3·7 |