Still's disease systemic JIA
Conditions
Interventions
Tapering and discontinuation of treatment if patients on rIL- 1RA therapy at
D90 (after 3 months of therapy) show a good clinical response (adapted ACRPed
score 90 or clinically inactive disease) an
Sponsors
Universitair Medisch Centrum Utrecht
Eligibility
Age
2 Years to 17 Years
Inclusion criteria
Inclusion criteria: Open label lead-in (observational part): 1. Children and adolescents diagnosed with sJIA (ILAR 2004 classification criteria);;2. Both male and female patients, aged 8 months - 16 years (anakinra is approved in children aged 8 months and older who suffer from CAPS, and as per definition, JIA has an onset before the age of 16); ;3. Parents or legal guardian (and the subject when age is appropriate) who are willing to sign the consent/assent forms.;Intervention part (tapering and stop phase): 1. patients treated with rIL-1RA as first line therapy showing an initial beneficial response (no fever on day 7) to rIL-1RA monotherapy (concomitant NSAID allowed); 2. Achieving at least an ACRPed90 response without fever around point 90 days after start of therapy on rIL-1RA mono therapy (concomitant NSAID allowed).
Exclusion criteria
Exclusion criteria: Open label lead-in (observational part): 1. An onset of Macrophage Activation Syndrome (MAS) simultaneously with sJIA or after the diagnosis of sJIA will lead to exclusion of a (potential) subject from participation in this study;;2. Previous systemically administered corticosteroid treatment within 6 weeks before diagnosis and enrollment.;3. Known exclusion criteria for the use of rIL-1RA (renal failure, with a creatinin clearance rate of
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| * The total (mean/median) number of injections of anakinra per patient during the first year of treatment; | — |
Secondary
| Measure | Time frame |
|---|---|
| * The number of patients with *clinically inactive disease* without medication at time point 1 year. * The total number of disease flares during or after tapering and stop of therapy in the first year; * The number of patients with remission off medication at time point 2 years; * The number of patients needing to switch treatment because of treatment failure during the first year (to calculate reduction in treatment costs) * The number of (serious) adverse events in the first year. | — |
Countries
Netherlands
Outcome results
None listed