Table 1.
Comparison of demographic data and family medical history between patients with extreme periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (ePFAPA) and non-extreme PFAPA (nPFAPA)
| Variables | ePFAPA | nPFAPA | P value |
|---|---|---|---|
| Male sex, n (%) | 31/47 (66%) | 173/318 (54%) | 0.14 |
| Mediterranean ancestry, n (%) | 21/42 (50%) | 149/299 (50%) | 0.98 |
| Sephardic, n (%) | 20/42 (48%) | 105/299 (35%) | 0.12 |
| Arabic, n (%) | 1/42 (2%) | 44/299 (15%) | 0.027 |
| Ashkenazi ancestry, n (%) | 1/42 (2%) | 12/299 (4%) | > 0.99 |
| Multiethnic ancestry, n (%) | 20/42 (48%) | 138/299 (46%) | 0.86 |
| Consanguinity, n (%) | 0 (0%) | 6/296 (2%) | > 0.99 |
| Family history of presumed PFAPA*, n (%) | 15/44 (34%) | 95/298 (32%) | 0.76 |
| Family history of FMF, n (%) | 14/43 (33%) | 66/304 (22%) | 0.11 |
| Family history of tonsillectomy, n (%) | 5/28 (18%) | 25/203 (12%) | 0.39 |
* Based on a familial history of recurrent fevers with signs of aphthous stomatitis, pharyngitis, and cervical adenitis in at least one first-degree relative
FMF, familial Mediterranean fever
The ePFAPA group comprised 47 patients. The nPFAPA group comprised 318 patients. The proportions and the percentages reflect the missing data