Table 4.
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.