Patients’ characteristics and therapeutic response according to the set of criteria fulfilled
| Overall (n=34) | Cantarini's criteria (n=6) | Vanoni's criteria (n=17) | PRINTO 2019 criteria (n=13) | Not classified* (n=13) | |
| Female/male | 23/11 | 4/2 | 12/5 | 8/5 | 9/4 |
| Age at onset (years) | 5.0 (2.4–15.8) | 21.5 (15.8–25.3) | 5.0 (1.5–9.0) | 2.5 (0.65–5.0) | 7.0 (4.5–16.0) |
| PFAPA duration (years) | 16.4 (10.0–20.4) | 4.5 (3.8–5.8) | 14.9 (6.8–18.2) | 16.4 (12.5–19.4) | 18.6 (12.5–20.5) |
| Annual frequency of flares before start of therapy | 12.0 (6.5–12.0) | 10.0 (5.8–12.0) | 12.0 (10.0–12.0) | 12.0 (9.3–14.8) | 8.3 (3.3–12.0) |
| Length of flares before start of therapy (days) | 3.8 (3.0–4.6) | 4.0 (3.9–4.8) | 4.0 (3.3–4.6) | 4.3 (3.3–5.1) | 3.0 (2.6–3.5) |
| CRP during flares (mg/L) | 113 (53–150) | 152 (59–201) | 104 (57–140) | 111 (60–144) | 67 (38–128) |
| Acute treatment of flares | |||||
| NSAID (n) | 7 | 0 | 5 | 4 | 1 |
| Corticosteroids (n) | 24 | 5 | 13 | 11 | 8 |
| Anakinra (n) | 5 | 3 | 3 | 2 | 1 |
| Long-term treatment | |||||
| Colchicine (n) | 29 | 5 | 14 | 10 | 12 |
| CR (%) | 19 | 0 | 9 | 14 | 38 |
| PR (%) | 38 | 50 | 36 | 29 | 38 |
| NR (%) | 29 | 50 | 27 | 43 | 13 |
| AE (%) | 19 | 0 | 27 | 14 | 13 |
| Anakinra (n) | 1 | 0 | 1 | 0 | 0 |
| CR (%) | 100 | – | 100 | – | – |
| PR (%) | 0 | – | 0 | – | – |
| NR (%) | 0 | – | 0 | – | – |
| Canakinumab (n) | 1 | 1 | 0 | 0 | 0 |
| CR (%) | 0 | 0 | – | – | – |
| PR (%) | 0 | 0 | – | – | – |
| NR (%) | 100 | 100 | – | – | – |
| Tonsillectomy/adenoidectomy (n) | 11 | 1 | 6 | 4 | 4 |
| CR/PR (%) | 27 | 0 | 17 | 25 | 50 |
| NR (%) | 73 | 100 | 83 | 75 | 50 |
| Outcome at last follow-up | |||||
| CR/PR with no treatment (%) | 50 | 40 | 56 | 67 | 40 |
Continuous variables are given in median (IQR).
*Patients fulfilling only modified Marshall’s criteria.
AE, adverse effect leading to discontinuation; CR, complete response;CRP, C-reactive protein; NR, no response; NSAID, nonsteroidal anti-inflammatory drug;PFAPA, periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis; PR, partial response.