Table 5 Comparison of clinical features of patients with extreme periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (ePFAPA), between those who met these criteria after steroid treatment (erPFAPA) and those who met the criteria upon first presentation
Variables | erPFAPA | ePFAPA upon presentation | P value |
|---|---|---|---|
Age at symptom onset (years), Median (IQR), N = 41 | 1.67 (1.0–2.5) | 1.5 (0.5–2.25) | 0.42 |
Age at diagnosis (years), Median (IQR), N = 44 | 2.25 (2.0–3.38) | 3.0 (1.5–4.25) | 0.75 |
Duration of flare (days), Median (IQR), N = 29 | 4.5 (3.5–6.75) | 3.0 (2.5–3.75) | 0.047 |
Initial interval between flares (weeks), Median (IQR), N = 41 | 3.5 (3.0–4.0) | 1.5 (1.0–2.0) | < 0.001 |
Intervals between flares after initiation of abortive treatment with corticosteroids (w), Median (IQR), N = 36 | 1 (1.0–1.5) | 1 (1–1.38) | 0.32 |
Pharyngitis (%) | 23/25 (92%) | 21/22 (95%) | > 0.99 |
Adenitis (%) | 12/25 (48%) | 12/22 (55%) | 0.65 |
Aphthous stomatitis (%) | 7/25 (28%) | 6/22 (27%) | 0.96 |
Abdominal pain (%) | 13/25 (52%) | 10/22 (45%) | 0.65 |
Headache (%) | 4/25 (16%) | 2/22 (9.1%) | 0.67 |
Myalgia (%) | 8/25 (32%) | 7/22 (32%) | > 0.99 |
Arthralgia (%) | 5/25 (20%) | 3/22 (14%) | 0.71 |
Rash (%) | 1/25 (4%) | 0 (0%) | > 0.99 |
Colchicine response | 7/15 (47%) | 9/10 (90%) | 0.04 |