Table 5.
Reviewed disease classification (selected cohort of patients; n = 64).
| Selected population (n = 64) | N (%) |
|---|---|
| Non-hematological disorders, n = 43 (67.2%) | |
| SLE | 3 (4.7%) |
| Cq1 deficiency (SLE) | 1 (1.6%) |
| GPA /Wegener's granulomatosis | 1 (1.6%) |
| Behcet's disease | 1 (1.6%) |
| RA | 1 (1.6%) |
| JIA | 7 (10.9%) |
| NMO | 4 (6.3%) |
| Crohn's disease | 5 (7.8%) |
| Other IBD | 4 (6.3%) |
| IPEX syndrome | 1 (1.6%) |
| Multi-systemic inflammatory disease syndrome | 3 (4.7)% |
| Lichen myxoedematosus | 1 (1.6%) |
| MKD/TRAPS polymorphism | 1 (1.6%) |
| Glanzmann's Thrombasthenia | 1 (1.6%) |
| Njimegen Breakage Syndrome | 1 (1.6%) |
| Tricohepatoenteric syndrome | 1 (1.6%) |
| Other Ads | 7 (10.9%) |
| Hematological disorders, n = 21 (32.8%) | |
| ITP | 2 (3.1%) |
| AIHA | 7 (10.9%) |
| Evans syndrome | 9 (14.1%) |
| Severe B-, T-, NK-cell immunodeficiency | 1 (1.6%) |
| SCID | 1 (1.6%) |
| PWCA | 1 (1.6%) |
The table includes details about disease characteristics of the selected cohort of patients with more detailed characteristics (n = 64), divided as “hematological disorders” and “non-hematological disorders.” In view of the spectrum of pediatric ADs, all diagnoses were reviewed and classified according to the study questionnaire. AIHA, autoimmune hemolytic anemia; ITP, immune thrombocytopenia; JIA, juvenile idiopathic arthritis; SCID, severe combined immuno deficiency; IPEX, immune dysregulation, polyendocrinopathy, enteropathy, X-linked; GPA, granulomatosis with polyangiitis; NMO, neuromyelitis optica; RA, rheumatoid arthritis; PWCA, pure white cell aplasia; TRAPS, TNF receptor-associated periodic syndrome; IBD, inflammatory bowel disease; SLE, systemic lupus erythematosus; ADs, autoimmune diseases; MKD, mevalonic kinase deficiency.