Table 2 Characteristics of fever episodes, family history and frequency of different symptoms during fever episodes
All PFAPA patients (n = 336) | PFAPA without atypical symptoms (n = 287) | PFAPA with atypical symptoms (n = 49) | |||
|---|---|---|---|---|---|
< 5 years (n = 252) | ≥ 5 years (n = 35) | < 5 years (n = 39) | ≥ 5 years (n = 10) | ||
Age of onset, median (range) | 2.0 (0.1–16) | 2.0 (0.1–4.9) | 6.5 (5–16) | 1.9 (0.1–4.5) | 9.2 (5–14) |
Distribution females/males | 46% / 54% | 47% / 53% | 46% / 54% | 44% / 56% | 40% / 60% |
Length (days) of fever episodes, median (range) | 4.5 (1.5–9) | 4.5 (1.5–6.5) | 3.8 (1.5–5.5) | 4.5 (2.5–9) | 5 (2.5–7) |
Interval (days) between episodes, median (range) a | 28 (11–84) | 28 (11–70) | 28 (14–84) | 28 (14–56) | 28 (16–31) |
Family history | |||||
Recurrent fevers | 28% | 27% | 34% | 28% | 30% |
Recurrent fevers in 1st degree relative | 20% | 20% | 23% | 18% | 10% |
Tonsillectomy | 25% | 25% | 26% | 21% | 50% |
Tonsillectomy in 1st degree relative | 13% | 14% | 3% | 15% | 20% |
Pharyngitisb | 94% | 95% | 100% | 95% | 60% |
Aphthous stomatitis | 43% | 44% | 34% | 46% | 60% |
Cervical adenitis | 81% | 81% | 71% | 90% | 80% |
Gastrointestinal (GI) symptoms | |||||
Any GI symptom, excl diarrhea | 46% | 44% | 40% | 54% | 50% |
Abdominal pain | 33% | 33% | 31% | 36% | 30% |
Nausea/vomiting | 17% | 17% | 11% | 23% | 20% |
Myalgia/arthralgia | 33% | 31% | 34% | 36% | 70% |
Headache | 15% | 13% | 23% | 18% | 30% |
Neurological symptoms | |||||
Dizziness | 1% | 0.4% | 3% | 3% | 0 |
Febrile seizures | 3% | 4% | 0 | 3% | 0 |