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. 2015 Feb 15;10:19. doi: 10.1186/s13023-015-0228-7

Table 4.

Canakinumab efficacy (used in 25 patients)

Disease Patient no. First anti–IL-1 Dose and frequency Clinical response Treatment duration (d) Associated treatment CCS DMARDs Still treated
AOSD 1 No 150 mg/4 wk No 148 No No No No
AoSD 2 No 150 mg/8 wk Total 31 Yes Yes No Yes
sJIA 3 No 4 mg/kg/4 wk Partial 61 Yes Yes Yes No
sJIA 4 No 150 mg/8 wk Total 499 UK UK UK No
sJIA 5 No 150 mg/4 wk No 31 Yes Yes Yes No
sJIA 6 No 4 mg/kg/4 wk Partial 28 Yes Yes No No
sJIA 7 No 3 mg/Kg/4 wk No 28 Yes Yes No No
sJIA 8 No 4 mg/kg/4 wk No 28 Yes Yes No No
sJIA 9 Yes 4 mg/kg/4 wk Partial 538 Yes Yes Yes No
FMF 10 No 2 mg/kg/8 wk Partial 731 Yes# No No Yes
FMF 11 No 2 mg/kg/8 wk Partial 1151 Yes# No No Yes
FMF 12 No 2 mg/kg/8 wk Total 589 Yes# No No Yes
FMF 13 Yes 2 mg/Kg on demand Total 966 Yes# No No Yes
MKD 14 Yes 2 mg/kg/8 wk Partial 802 Yes Yes No Yes
MKD 15 Yes 2-3* mg/kg/8 wk Partial 1112 No No No Yes
MKD 16 No 3 mg/kg/8 wk Total 1101 UK UK UK Yes
MKD 17 No 2 mg/kg/8 wk Total 957 No No No Yes
MKD 18 No 7 mg/kg/8 wk Partial 558 Yes** No No Yes
MKD 19 No 3.5 mg/kg/8 wk Total 163 UK UK UK Yes
Vasculitis 20 No 150 mg/8 wk Partial 181 Yes Yes No No
Vasculitis 21 No 150 mg/8 wk UK UK UK UK UK No
Schnitzler’s syndrome 22 No 150 mg/8 wk Partial 385 No No No Yes
TRAPS 23 No 2 mg/kg/4 wk Total 170 Yes*** No No Yes
Erdheim Chester 24 No 2 mg/kg/8 wk Partial 679 Yes Yes No Yes
Blau syndrome 25 No 150 mg/8 wk Total 390 Yes Yes No Yes

AOSD: adult onset Still’s disease; sJIA: systemic juvenile idiopathic arthritis; FMF: familial Mediterranean fever; MKD: mevalonate kinase deficiency, Vasculitis: giant cell arteritis (2) and polyarteritis nodosa (2), TRAPS: tumor necrosis factor receptor-associated periodic syndrome.

#FMF: all patients received colchicine with canakinumab.

*MKD patient no. 15: initial dose 2 mg/kg, then augmented to 3 mg/Kg.

*MKD patient no. 18: adalimumab was associated at the time of canakinumab onset.

***TRAPS: enalapril was given with canakinumab.

CCS: corticosteroids.