Table 2 Comparison of clinical features between patients with extreme periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (ePFAPA) and non-extreme PFAPA (nPFAPA)
Variables | ePFAPA | nPFAPA | P value |
|---|---|---|---|
Age at symptom onset (years), Median (IQR), N = 329 | 1.5 (0.7–2.5) | 2.5 (1.5-4.0) | < 0.001 |
Age at diagnosis (years), Median (IQR), N = 3 18 | 2.6 (2.0–3.6) | 4.5 (3.0–6.2) | < 0.001 |
Duration of flare (days), Median (IQR), N = 239 | 3.5 (2.5–5.0) | 4.0 (3.0–5.5) | 0.86 |
Initial interval between flares (weeks), Median (IQR), N = 330 | 2.0 (1.5–3.5) | 4.0 (2.5–4.5) | < 0.001 |
Intervals between flares after initiation of abortive treatment with corticosteroids (weeks), Median (IQR), N = 192 | 1.0 (1.0–1.5) | 2.5 (1.9–4.9) | < 0.001 |
Maximal fever (°C), Median (IQR), N = 134 | 40.0 (39.6–40.0) | 40.0 (39.2–40.0) | 0.35 |
Pharyngitis (%) | 44/47 (94%) | 290/309 (94%) | > 0.99 |
Adenitis (%) | 24/47 (51%) | 112/307 (36%) | 0.056 |
Aphthous stomatitis (%) | 13/47 (28%) | 72/309 (23%) | 0.51 |
Abdominal pain (%) | 23/47 (49%) | 142/309 (46%) | 0.7 |
Headache (%) | 6/47 (13%) | 45/308 (15%) | 0.74 |
Myalgia (%) | 15/47 (32%) | 79/308 (26%) | 0.36 |
Arthralgia (%) | 8/47 (17%) | 50/308 (16%) | 0.89 |
Rash (%) | 1/47 (2%) | 14/318 (4%) | 0.7 |