Table 3 Outcome in CAPS patients treated with canakinumab
Patients | Phenotype | age at diagnosis (months) | the start age of treatment (months) | initial manifestations before treatment | manifestations after treatment |
|---|---|---|---|---|---|
1 | FCAS | 125 | 125 | Febrile attacks, urticaria-like rash, slightly papilledema, WBC/CRP↑ | No |
2 | MWS | 22 | 32 | Febrile attacks, urticaria-like rash, arthralgia, WBC/CRP/SAA↑ | Occasional rash |
3 | MWS | 125 | 135 | Febrile attacks, urticaria-like rash, arthralgia and joint swelling, CRP/SAA↑ | occasionally arthralgia, intermittent CRP↑ESR↑ |
4 | MWS | 155 | 155 | Febrile attacks, urticaria-like rash, intermittent arthralgia, slightly decreased auditory threshold, WBC/CRP/SAA↑ | No |
5 | CINCA | 4 | 14 | Febrile attacks, urticaria-like rash, occassional right limb pain, decreased auditory threshold, sterile meningitis, WBC/CRP/SAA↑ | Occasional rash and intermittent fever,WBC/CRP↑frequently |
6 | CINCA | 5 | 26 | Febrile attacks, urticaria-like rash, optic neuritis, sterile meningitis and hydrocephalus, WBC/CRP/SAA↑ | Occasional fever, hydrocephalus |
7 | CINCA | 13 | 25 | Febrile attacks, urticaria-like rash, arthralgia and joint swelling, sterile meningitis and hydrocephalus, WBC/CRP/SAA↑ | synovial enhancement, hydrocephalus |
8 | CINCA | 6 | 6 | Febrile attacks, urticaria-like rash, decreased auditory threshold, sterile meningitis and abnormalities in thalamus, WBC/CRP↑ | Abnormalities in thalamus |
9 | CINCA | 5 | 15 | Febrile attacks, urticaria-like rash, sterile meningitis, papilledema, WBC/CRP↑ | No |
10 | CINCA | 2 | 18 | Febrile attacks, urticaria-like rash, optic neuritis, decreased auditory threshold, WBC/SAA↑ | slightly decreased auditory threshold |