Table 1

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
Male30 (50%)32 (53%)26 (43%)35 (58%)28 (47%)28 (47%)
Paediatric/Adults54/633/2745/1529/3159/139/21
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.