Table 1.
Demographic and clinical features of patients diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome (PFAPA group) and patients with fever of unknown origin (control group).
| PFAPA group | Control group | |
|---|---|---|
| Age (years) | 34.00 ± 11.86 | 40.56 ± 16.45 |
| Males (%)/females (%) | 48 (64.9)/26 (35.1) | 23 (37.1)/39 (62.9) |
| Age at disease onset (years) | 26.55 ± 10.03 | 27.94 ± 17.67 |
| Mean temperature at attacks (°C) | 39.31 ± 0.92 | 38.9 ± 1.02 |
| Attacks per year | 15.2 ± 8.44 | 9.45 ± 7.03 |
| Duration of flares | ||
| ≤2 days | 2 (2.7%) | 14 (22.6%) |
| 3–5 days | 46 (62.2%) | 12 (18.2%) |
| 6–9 days | 9 (12.2%) | 8 (12.9%) |
| ≥10 days | 11 (14.9%) | 22 (35.5%) |
| PFAPA cardinal symptoms during attacks | ||
| Pharyngitis | 70 (94.6%) | 39 (62.9%) |
| Cervical lymphadenitis | 61 (82.4%) | 14 (22.6%) |
| Oral aphthosis | 48 (64.9%) | 21 (33.9%) |
| Other associated symptoms during attacks | ||
| Generalized lymphadenitis | 4 (5.4%) | 14 (22.6%) |
| Asthenia | 62 (83.8%) | 51 (82.3%) |
| Abdominal pain | 33 (44.6%) | 20 (32.3%) |
| Diarrhea and/or vomiting | 16 (21.6%) | 13 (21%) |
| Thoracic pain | 13 (17.6%) | 24 (38.7%) |
| Arthralgia | 53 (71.6%) | 42 (67.7%) |
| Arthritis | 11 (14.9%) | 16 (25.8%) |
| Myalgia | 47 (63.5%) | 37 (59.7%) |
| Urticaria-like rash | 4 (5.4%) | 10 (16.1%) |
| Erythematous rash | 9 (12.2%) | 0 (0.0%) |
| Erysipelas-like rash | 0 (0.0%) | 3 (4.8%) |
| Maculo-papular rash | 3 (4.1%) | 9 (14.5%) |
| Periorbital edema | 6 (8.1%) | 5 (8.1%) |
| Conjunctivitis | 8 (10.8%) | 18 (29%) |
| Headache | 43 (58.1%) | 37 (59.7%) |
| Genital aphthosis | 3 (4.1%) | 2 (3.2%) |
| Increased inflammatory markers during attacks | 72 (97.3%) | 46 (74.2%) |
| Symptom-free intervals | 72 (97.3%) | 33 (53.2%) |
Quantitative data are referred as mean ± SD values; qualitative data are reported as frequency counts and percentages.