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Phase 3 N=47 Randomized Quadruple-blind Treatment

Rilonacept for Treatment of Cryopyrin-Associated Periodic Syndromes (CAPS)

Familial Cold Autoinflammatory Syndrome (FCAS) · Familial Cold Urticaria · Muckle-Wells Syndrome (MWS) · Genetic Diseases, Inborn

Enrolled (actual)
47
Serious AEs
5.5%
Results posted
Nov 2009
Primary outcome: Primary: Change From Baseline to Week-6 (Part A) Endpoint in Mean Key Symptom Score (KSS) — 2.4; 3.1; 2.1; 0.5 Units of a Scale — p=<.0001

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
rilonacept 160 mg (Drug); Placebo (Drug)
Age
Pediatric, Adult, Older Adult · 7+ yrs
Sex
All
Sponsor
Regeneron Pharmaceuticals
Primary completion
Jun 2008

Outcome Measures

OutcomeResultp-value
PRIMARY
Change From Baseline to Week-6 (Part A) Endpoint in Mean Key Symptom Score (KSS)
2.4; 3.1; 2.1; 0.5; -0.3; -2.6 <.0001 sig
PRIMARY
Mean Change in Key Symptom Score (KSS) From Week 15 to Week 24 (During the Randomized Withdrawal Phase or Part B)
0.2; 0.3; 1.2; 0.4; 0.9; 0.1 <0.001 sig

Summary

Inflammatory symptoms of Cryopyrin-Associated Periodic Syndrome (CAPS) are due to mutations in a the NLRP-3 gene (previously known as Cold Induced Autoinflammatory Syndrome-1 or CIAS1). These mutations result in the body's overproduction of interleukin-1 (IL-1), a protein that stimulates the inflammatory process. IL-1 Trap (rilonacept) was designed to bind to the interleukin-1 cytokine and prevent it from binding to its receptors in the body.

Eligibility Criteria

Inclusion Criteria

  • Double-blind phases: adults age 18 and above; Open-label extension: Adults and children aged 7 years and older.
  • Was diagnosed with Familial Cold Auto-inflammatory Syndrome (FCAS) or Muckle-Wells Syndrome (MWS) based upon clinical signs and symptoms
  • Had documented mutation in NLRP-3 (Cold Induced Autoinflammatory Syndrome-1 or CIAS1) in subject or relative, and willingness to have a confirmatory genetic (Deoxyribonucleic acid or DNA) test (cheek swab).
  • Was able to understand and comply with study procedures and was able to provide informed consent
  • If female, was not currently pregnant and was willing to use contraception during the study

Exclusion Criteria

  • Had evidence of untreated tuberculosis or other conditions/therapies that made the subject inappropriate for this study.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT00288704). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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