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Phase 3 N=17 Treatment

Efficacy, Safety and Tolerability of ACZ885 in Pediatric Patients With the Following Cryopyrin-associated Periodic Syndromes: Familial Cold Autoinflammatory Syndrome, Muckle-Wells Syndrome, or Neonatal Onset Multisystem Inflammatory Disease

Cryopyrin-associated Periodic Syndromes · Familial Cold Autoinflammatory Syndrome · Muckle-Wells Syndrome · Neonatal Onset Multisystem Inflammatory Disease

Enrolled (actual)
17
Serious AEs
23.5%
Results posted
Aug 2015
Primary outcome: Primary: Percentage of Participants Aged 4 Years or Younger With at Least One Complete Response at Week 56 — 94.1 Percentage of participants

Study Design & Population

Study type
Interventional
Phase
Phase 3
Interventions
ACZ885 (Drug)
Age
Pediatric · 0+ yrs
Sex
All
Sponsor
Novartis Pharmaceuticals
Primary completion
Nov 2014

Outcome Measures

OutcomeResultp-value
PRIMARY
Percentage of Participants Aged 4 Years or Younger With at Least One Complete Response at Week 56
94.1
SECONDARY
Percentage of Participants Aged 2 Years or Younger With at Least One Complete Response at Week 56
90
SECONDARY
Percentage of Participants With Defined Grades in Physician's Global Assessment Score at Week 56
70.6; 23.5; 0; 5.9; 0
SECONDARY
Percentage of Participants With Defined Grades in Physician Assessment of Skin Disease at Week 56
82.4; 5.9; 11.8; 0; 0
SECONDARY
Change From Baseline in C--Reactive Protein (CRP) and Serum Amyloid A (SAA) Concentrations at Week 56
-5.4; -54.4
SECONDARY
Number of Participants With Adverse Events (AEs) and Serious Adverse Events (SAEs)
17; 4; 10; 3
SECONDARY
Percentage of Participants Receiving a Concomitant Vaccination During the Study
41.2
SECONDARY
Number of Vaccination Cases With Protective Antibody Levels Following Immunization With Inactivated Vaccines
18; 13
SECONDARY
Number of Participants With Anti-canakinumab Antibodies at Week 56

Summary

This trial will assess the safety, efficacy and tolerability of ACZ885 in patients aged 4 years and younger with cryopyrin associated periodic syndromes (CAPS)

Eligibility Criteria

Inclusion Criteria

  • Male and female patients that are 28 days up to 60 months of age at the time of the screening visit.
  • Body weight > or = 2.5 kg.
  • Parent or legal guardian's written informed consent is required before any assessment is performed for patients.
  • At study entry, patients should have a clinical diagnosis of FCAS, MWS, or NOMID and symptoms requiring pharmacological intervention. Prior agreement between the Investigator and Novartis for study eligibility is required for patients who do not have a molecular diagnosis of NALP3 mutations available (either testing not performed, or testing performed but negative) upon study entry. For those patients who have not been molecularly tested for NALP3 mutations, molecular testing should be performed during the course of the study.
  • For patients treated with an IL-1 blocking agent (i.e. anakinra, rilonacept), these treatments should be discontinued prior to the baseline visit and patients must demonstrate active disease prior to treatment.
  • Patients who are scheduled to receive an immunization, according to their local vaccination guidelines, with an inactivated vaccine must be willing to participate in the assessment schedule for vaccinated patients.

Exclusion Criteria

  • Preterm neonates for whom, in the Investigator's judgment, participation in the study is not deemed appropriate.
  • History of recurrent and/or evidence of active bacterial, fungal, or viral infections (including HIV).
  • Patients with immunodeficiency or treatment with immunosuppressive drugs.
  • Live vaccinations within 6 months with a positive PPD test [defined as an induration of at least 10mm])
  • Patients with end-stage renal disease
  • Patients with diabetes mellitus
  • Patients receiving immunosuppressive therapy
  • Patients with hematologic cancers.
  • Participation in another trial within the last 30 days or 5 half-lives of the investigational compound (whichever is longer).
  • Familial and social conditions rendering regular medical assessment not possible.
  • Pediatric patients with neutropenia (absolute neutrophil count [ANC] < 1.5 x 10 to the 9th/l)

Other protocol defined inclusion/exclusion criteria may apply

View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT01302860). 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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