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Table 1 Demographics and baseline disease characteristics

From: Canakinumab (ACZ885, a fully human IgG1 anti-IL-1β mAb) induces sustained remission in pediatric patients with cryopyrin-associated periodic syndrome (CAPS)

Patient Clinical picture/NALP3mutation - Clinical symptom Weight (kg) CRP (mg/L) SAA (mg/L) Previous anakinra use/response Physician's global assessment of disease activity
1 MWS/V198M -
pyrexia, conjunctivitis, headache, abdominal pain, arthralgia and lassitude
17.2 8.2 1.8 Yes/No* Moderate
2 MWS/E311K-
aphthae, stomach pain, fatigue, loss of energy, headache, conjunctivitis, myalgia, fever peaks, beginning of hearing deficit and arthralgia during night.
18.1 2.0 2.1 Yes/Partial** Moderate
3 MWS/T348M-
23.3 39.0# 74.0# Yes/Yes Moderate
4 MWS/V198M -
Low level of immunoglobulins, pyrexia, sensitivity to infection, coldness exposed exanthema, conjunctivitis, headache, oral aphthae, abdominal pain, myalgia and fatigue.
24.1 0.2 2.9 Yes/Yes Moderate
5 MWS/E311K -
exanthema, myalgia, conjunctivitis, attention deficit, headache, lack of concentration, oral aphthae, fatigue and hearing deficiency
35.3 9.9 14.1 No Moderate
6† NOMID/T348M -
urticarial rash, hepatomegaly, pyrexia, anemia, headache, stomach pain, malaise, nausea, fatigue, conjunctivitis, high frequency hearing loss (1 KHz), sterile meningitis, papilloedema, growth retardation, bilateral reduced visual acuity, knee arthritis, back pain, myalgia, leucocytosis, and thrombocytosis
48.6 38.9 198.0 Yes/Yes Moderate
7† NOMID/G569R -
exanthema, papillar edema, pseudo-tumor cerebri, hearing loss, arthritis, enlarged inner and outer liquor cavities, morphologically elevated bilateral intraocular pressure, and an enlarged blind spot in the visual field
52.0 65.6 151.0 Yes/Yes Moderate
  1. CRP, C-reactive protein; SAA, Serum Amyloid A
  2. # Local lab values
  3. †The two adolescent patients had MWS/NOMID
  4. *No response - lack of efficacy while on anakinra
  5. **Partial response - MWS activity despite of anakinra therapy and necessity of anakinra dosage increase