Patient | Age at First Symptoms | Time to Abatacept* | Response Following Abatacept | Total Months of Abatacept Therapy | Treatment Regimen at Abatacept Initiation† | Treatment Regimen at Last Followup† |
---|---|---|---|---|---|---|
1 | 8 mo | 46 mo | Fever improved; ESR normalized; requires dosing every 3 wks for greater arthritis control | 17 | Ank 11 mg/kg/day CS 1.1 mg/kg/day MTX 0.8 mg/kg/wk | Ank 0.9 mg/kg/day CS 0.9 mg/kg/day MTX 0.7 mg/kg/wk Abt 18 mg/kg every 3 wks |
2 | 10 yrs | 18 mo | Stopped prednisone at 2 mo; cushingoid symptoms have resolved; no disease flares or MAS | 8 | Ank 8.9 mg/kg/day CS 4.2 mg/kg/day Cyclosporine 4.4 mg/kg/day IV CYC 466 mg/m2 every 4 wks IVIG 1.7 g/kg every 3 wks | Ank 2.3 mg/kg/day CS 0.2 mg/kg/day Abt 18 mg/kg every 4 wks |
3 | 2 yrs | 66 mo | Improved arthritis; ESR began to normalize at 2 mo | 13 | Ank 5.1 mg/kg/day CS 0.8 mg/kg/day MTX 0.9 mg/kg/wk | Ank 3.5 mg/kg/day CS 0.5 mg/kg/day MTX 0.6 mg/kg/wk Abt 10 mg/kg every 4 wks |
4 | 2 yrs | 40 mo | Improved arthritis; ESR began to normalize at 3 mo | 9 | Ank 11 mg/kg/day CS 0.8 mg/kg/day MTX 0.8 mg/kg/wk | Ank 8.4 mg/kg/day CS 0.6 mg/kg/day Abt 13 mg/kg every 4 wks |
↵* Months since onset of initial sJIA symptoms to start of abatacept.
↵† Corticosteroid dosing was converted into prednisone equivalent and calculated as mg/kg/day. Ank: anakinra; CS: corticosteroids; MTX: methotrexate (subcutaneous); Abt: abatacept; IVIG: intravenous immunoglobulin; MAS: macrophage activation syndrome; ESR: erythrocyte sedimentation rate; sJIA: systemic juvenile idiopathic arthritis; CYC: cyclophosphamide.