Autoantibodies in patients with hypomorphic RAG mutations
| Patients | n | n (%) with autoantibodies | Specificity |
| RAG deficiency | |||
| OS/LS | 14 | 9 (64.3) | Five ANA, four TPO, two pANCA, one TG, and one OmpC |
| T− B− SCID | 5 | 0 (0) | |
| SCIDX1 | 14 | 0 (0) | |
| Controls | 6 | 1 (16.7) | ANA |
| Patients | n | n (%) with autoantibodies | Specificity |
| RAG deficiency | |||
| OS/LS | 14 | 9 (64.3) | Five ANA, four TPO, two pANCA, one TG, and one OmpC |
| T− B− SCID | 5 | 0 (0) | |
| SCIDX1 | 14 | 0 (0) | |
| Controls | 6 | 1 (16.7) | ANA |
OS, Omenn syndrome; LS, leaky SCID; TPO, thyroid peroxidase; pANCA, perinuclear anti-neutrophil cytoplasmic antibody; TG, thyroglobulin; and OmpC, outer membrane porin protein C.