Demographic features of the 360 patients included in the study
FMF n=60 | CAPS n=60 | MKD n=60 | TRAPS n=60 | PFAPA n=60 | uRF n=60 | |
Male | 30 (50%) | 32 (53%) | 26 (43%) | 35 (58%) | 28 (47%) | 28 (47%) |
Paediatric/Adults | 54/6 | 33/27 | 45/15 | 29/31 | 59/1 | 39/21 |
Age, years, median (range) | 10.5 (7.0–15.5) | 16.0 (8.9–31.6) | 16.2 (9.1–23.0) | 21.9 (10.5–41.1) | 6.6 (3.8–9.5) | 13.5 (8.2–26.4) |
Age at disease onset, median (range) | 3.4 (1.2–6.4) | 3.0 (0.5–11.2) | 0.4 (0.2–0.9) | 3.4 (0.8–10.6) | 1.5 (0.7–3.0) | 5.9 (2.0–19.1) |
Disease duration, median (range) | 5.6 (2.7–10.2) | 9.0 (4.6–19.1) | 14.2 (7.9–20.8) | 13.3 (6.8–23.2) | 3.9 (2.3–6.8) | 4.8 (3.0–8.2) |
Episode duration, median (range) | 3.0 (2.0–4.0) | 2.0 (0.8–5.0) | 5.0 (4.0–7.0) | 8.0 (5.0–18.0) | 4.0 (3.0–5.0) | 4.0 (3.0–7.0) |
Number episodes/year, median (range) | 12.0 (10.0–20.0) | 12.0 (6.0–25.0) | 12.0 (10.0–16.0) | 6.0 (4.0–12.0) | 12.0 (12.0–18.0) | 12.0 (5.0–13.0) |
CAPS, cryopyrin-associated periodic syndromes; FMF, familial Mediterranean fever; MKD, mevalonate kinase deficiency; PFAPA, periodic fever, aphthosis, pharyngitis and adenitis; TRAPS, tumour necrosis factor receptor-associated periodic fever syndrome; uRF, undefined recurrent fevers.