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Efficacy, Safety, and Tolerability of ACZ885 in Patients With Muckle-Wells Syndrome

Phase 3
Completed
Conditions
Muckle Wells Syndrome
Interventions
Drug: Placebo
Registration Number
NCT00465985
Lead Sponsor
Novartis
Brief Summary

This study is designed to provide efficacy and safety data for ACZ885 (a fully human anti-interleukin-1beta (anti-IL-1beta) monoclonal antibody) administered as an injection subcutaneously (s.c.) in patients with Muckle-Wells Syndrome.

Part I is an 8-week open-label, active treatment period to identify ACZ885 responders.

Part II is a double-blind, placebo-controlled period to assess primarily the efficacy of ACZ885 compared to placebo.

Part III is an open-label, active treatment period where patients will receive ACZ885 every 8 weeks after withdrawal or completion of Part II.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
35
Inclusion Criteria
  • Molecular diagnosis of NALP3 mutations and clinical picture resembling Muckle-Wells Syndrome.
  • Muckle-Wells Syndrome patients who participated in the CACZ885A2102 study, will have the option to participate in this study upon disease flare
  • Muckle-Wells Syndrome patients requiring medical intervention either untreated or treated (i.e. under ACZ885, anakinra, or any other investigational IL-1 blocking therapy).
Exclusion Criteria
  • History of being immunocompromised, including a positive HIV at screening test result.
  • No live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose.
  • History of significant medical conditions, which in the Investigator's opinion would exclude the patient from participating in this trial.
  • History of recurrent and/or evidence of active bacterial, fungal, or viral infections.
  • Positive tuberculin skin test at 48 to 72 hours after administration at the screening visit or within 2 months prior to the screening visit, according to national guidelines.

Other protocol-defined inclusion/exclusion criteria may apply

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Part II - arm 2Placebo-
Part I, Part II-arm1, & Part IIIACZ885-
Primary Outcome Measures
NameTimeMethod
Percent of Participants With Disease Flare in Part II (After 24 Weeks of the Double-blind Part)32 weeks after study start

Determined by the Physician's global assessment of autoinflammatory disease activity, assessment of skin disease and inflammation markers. Data expressed as a percent of participants who had experienced a flare by the end of Part II.

Number of Participants Who Experienced a Disease Flare in Part II32 weeks after study start

Disease flare is determined by the Physician's global assessment of autoinflammatory disease activity, assessment of skin disease and inflammation markers. Disease Flare = the C-reactive protein and/or serum amyloid A (SAA) \> 30 mg/L and either a PGA \> minimal, or PGA equal to minimal and \> minimal SD.

Secondary Outcome Measures
NameTimeMethod
Investigator's Clinical Assessment of Autoinflammatory Disease Activity & Participant's Assessment of Symptoms at End of Part II (After 24 Weeks of the Double-blind Part)32 weeks after study start

A 5-point scale was used for the Physician's global assessment on autoinflammatory disease activity (absent, minimal, mild, moderate and severe) and for the assessment of the following items:

* skin disease (urticarial skin rash)

* arthralgia

* myalgia

* headache/migraine

* conjunctivitis

* fatigue/malaise

* other symptoms related to autoinflammatory syndrome

* other symptoms not related to autoinflammatory syndrome

Change in Inflammation Markers at the End of Part II (C-reactive Protein and/or Serum Amyloid A) (After 24 Weeks of the Double-blind Part) From Week 8.Week 8 and Week 32
Pharmacokinetics (CLD (L/d))48 weeks after study start

Assessed serum clearance of ACZ885.

Pharmacodynamics Measured by Interleukin-1β (IL-1β) Concentrations at End of Part I.until Week 8
Number of Participants With Treatment Response in Part I (After 8 Weeks)8 weeks after study start

Treatment response was based on Physician's global assessment(PGA) of autoinflammatory disease activity, assessment of skin disease(SD) and serum values of C-reactive protein(CRP) and/or serum amyloid A(SAA). Complete Response (CR):PGA and SD ≤ minimal and normal CRP and/or SAA. Partial Response (PR): a reduction of CRP and/or SAA from baseline (BL) by \>30% but not reaching normal values and PGA improvement from BL by at least one category. Disease flare: a CRP and/or SAA \> 30 mg/L and either PGA \> minimal or PGA = minimal and SD \> minimal. Non-responders = no PR by Day 8 or no CR by Day 15.

Pharmacodynamics Measured by Interleukin-1β (IL-1β) Concentrations at End of Part II.32 weeks after study start
Pharmacodynamics Measured by Interleukin-1β (IL-1β) Concentrations at End of Part III.48 weeks after study start

Trial Locations

Locations (2)

Novartis Investigational Site

🇫🇷

Lille Cedex, France

Novartis Investigative Site

🇬🇧

London, United Kingdom

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