Table 1 Clinical characteristics of PAN and DADA2 patients at the time of diagnosis
PAN (n = 11) | DADA2 (n = 4) | |
|---|---|---|
Female/Male | 6/5 | 1/3 |
Age at symptom onset, median (min-max) | 4 (2–10) | 3.5 (1.5–14) |
Age at diagnosis median (min-max) | 6 (3–12) | 4 (3–15) |
Age at the OCT visit median (min-max) | 8 (4–16) | 6 (5–16) |
Constitutional symptoms, n (%) | 8 (73%) | 4 (100%) |
Musculoskeletal symptoms, n (%) | 10 (90%) | 3 (75%) |
Cutaneous involvement, n (%) | 8 (73%) | 3 (75%) |
Hypertension, n (%) | 0 (0%) | 1 (25%) |
Renal involvement, n (%) | 0 (0%) | 1 (25%) |
Neurologic involvement, n (%) | 1 (9%) | 0 (0%) |
Testis involvement, n (%) | 1 (9%) | 1 (25%) |
Gastrointestinal system involvement, n (%) | 9 (82%) | 3 (75%) |
Cardiovascular involvement, n (%) | 0 (0%) | 0 (0%) |
Pulmonary involvement, n (%) | 0 (0%) | 0 (0%) |
Medication n (%) | ||
Steroid | 11 (100%) | 4 (100%) |
Biologic agent | 1 (9%) | 4 (100%) |
DMARD | 8 (83%) | 0 (0%) |