Disease | PFAPA n=259 | MKD n=65 | CAPS n=86 | Unconfirmed BD n=63 | Confirmed BD n=156 | p Value* |
---|---|---|---|---|---|---|
Age, mean±SD | 6.95±3.42 | 8.73±4.11 | 8.88±4.24 | 11.91±3.82 | 13.25±3.88 | <0.0001 |
Oral aphthosis | 153 | 38 | 12 | 63 | 156 | <0.0001 |
Genital aphthosis | 0 | 1 | 0 | 20 | 91 | <0.0001 |
Ocular sign† | 1 | 2 | 6 | 12 | 67 | <0.0001 |
Ocular signs‡ | 1 | 2 | 23 | 12 | 73 | <0.0001 |
Neurologic signs | 42 | 25 | 35 | 15 | 72 | <0.0001 |
Neurologic signs¶ | 6 | 8 | 26 | 0 | 37 | <0.0001 |
Vascular symptoms | 0 | 1 | 0 | 2 | 27 | <0.0001 |
Data are no. (%) unless indicated.
*By χ2 test.
†Ocular sign: includes at least one of anterior and posterior uveitis and retinal vasculitis.
‡Ocular signs: includes at least one of anterior and posterior uveitis, retinal vasculitis and papilledema.
¶Neurological signs: calculated without headaches and with papilledema.
CAPS, cryopyrin-associated periodic syndrome; MKD, mevalonate kinase deficiency; PFAPA, periodic fever adenitis pharyngitis and aphthosis.