Table 4

Frequency of symptoms among diseases and confirmed and unconfirmed paediatric Behçet's disease (BD)

DiseasePFAPA
n=259
MKD
n=65
CAPS
n=86
Unconfirmed BD
n=63
Confirmed BD
n=156

p Value*
Age, mean±SD6.95±3.428.73±4.118.88±4.2411.91±3.8213.25±3.88<0.0001
Oral aphthosis153381263156<0.0001
Genital aphthosis0102091<0.0001
Ocular sign†1261267<0.0001
Ocular signs‡12231273<0.0001
Neurologic signs4225351572<0.0001
Neurologic signs¶6826037<0.0001
Vascular symptoms010227<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.