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Abstract Number: 1784

Effectiveness Of First Line Use Of Recombinant IL-1RA Treatment In Steroid naïve Systemic Juvenile Idiopathic Arthritis: Results Of a Prospective Cohort Study

S.J. Vastert1, Wilco de Jager2, Bo Jan Noordman2, Dirk Holzinger3, Wietse Kuis4, Berent J. Prakken2 and Nico M. Wulffraat5, 1Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands, 2Pediatric Immunology, University Medical Center Utrecht, Utrecht, Netherlands, 3Institute of Immunology, University Muenster, Muenster, Germany, 4Pediatric immunology, University Medical Center Utrecht, Utrecht, Netherlands, 5UMC Utrecht, Utrecht, Netherlands

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: anakinra and treatment, Systemic JIA

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Session Information

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects II: Autoinflammatory Disease and Systemic Juvenile Idiopathic Arthritis

Session Type: Abstract Submissions (ACR)

Background/Purpose: To perform a prospective cohort study with recombinant IL-1RA (Anakinra, rIL-1RA) as first-line therapy in newly onset sJIA.

Methods: Patients fulfilled the ILAR criteria for sJIA. RIL-1RA (2 mg/kg) was started as first-line therapy and patients were monitored clinically and immunologically. The protocol contained a stop strategy when in remission at 3 months.

Results: We included 20 consecutive newly onset sJIA patients and started rIL1-RA as first line treatment (in steroid naive patients). Mean follow-up was 32 months (range 12-54). After 3 months, 85% of patients showed ACRPed90 responses and 75% (15/20) were in remission on rIL-1RA alone. After 1 year, 85% of patients (17/20) displayed ACRPed90 responses / clinically inactive disease. 65% of patients (13/20) achieved this on rIL-1RA alone. However, 7/20 patients (35%) required therapy besides rIL1-RA because of persistent disease activity. Following our stop strategy, 11/15 (73%) patients with clinically inactive disease at 3 months, could stop rIL-1RA within the first year.

After two years, (n=14), 86% (12/14) of patients showed ACRPed90 responses / disease remission, either on (n=4) or off (n=8) medication. After three years (n=11), 91% of patients (10/11) showed ACRPed90 responses / disease remission, either on (n=2) or off (=8) medication.

Conclusion: This is the first prospective study describing rIL-1RA as first-line therapy in sJIA. We observed excellent clinical responses in nearly all patients within three months. The majority of responding patients could stop rIL-1RA within 1 year with preserved remission during follow-up. Approximately one-third of patients needed concomitant therapy to maintain clinical response. IL-18 and S100A proteins seem candidate biomarkers for guiding tapering.


Disclosure:

S. J. Vastert,

Novartis Pharmaceutical Corporation,

5;

W. de Jager,
None;

B. J. Noordman,
None;

D. Holzinger,
None;

W. Kuis,
None;

B. J. Prakken,
None;

N. M. Wulffraat,

Roche, Pfizer,

2,

Novartis Pharmaceutical Corporation,

5.

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