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NCT Number: NCT07598422

A Study to Assess the Effectiveness and Safety of Canakinumab in Clinical Use in Patients With Schnitzler's Syndrome

The aim of this study is to assess the effectiveness and safety of Ilaris (canakinumab) in clinical use in patients with Schnitzler's syndrome.

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Key information

Conditions

Schnitzler Syndrome Immune System Diseases Immunoproliferative Disorders Monoclonal Gammopathy of Undetermined Significance Paraproteinemias

Age range

0 year and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Novartis Investigative Site, Kanoya, Kagoshima-ken, Japan

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Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

Inclusion criteria

  • Patients diagnosed with Schnitzler's syndrome using canakinumab for the first time for the indication of Schnitzler's syndrome as described in the package insert.

Patients starting treatment with canakinumab before conclusion of the contract for this study will also be included in the study so that all patients diagnosed with Schnitzler's syndrome who received canakinumab will be registered in this study.

Exclusion criteria

  • Patients previously treated with canakinumab in Study IACT21071.
  • All patients treated with canakinumab for the following indications:

Indications: Cryopyrin-associated periodic syndromes (familial cold autoinflammatory syndrome, Muckle-Wells syndrome, neonatal onset multisystem inflammatory disease), hyper immunoglobulin D (IgD) syndrome (mevalonate kinase deficiency), tumor necrosis factor (TNF) receptor-associated periodic syndrome, familial mediterranean fever, systemic juvenile idiopathic arthritis, adult-onset Still's disease.

  • Patients receiving canakinumab for off-label use under the Clinical Trials Act or GCP (e.g., patient-proposed healthcare services, investigator-initiated clinical trial).

Treatment and study plan

Primary outcomes

  1. Proportion of Patients Achieving Complete Clinical Response in the Period From 7 Days to 8 Weeks After the First Dose

    Time frame: Day 8 to Week 8

    Complete clinical response is defined as a Physician's Global Assessment (PGA) score of ≤ 5, with none of 5 items (urticaria, fatigue, fever and chills, myalgia, and arthralgia and bone pain) rated as > 1.

    The PGA is a tool designed to evaluate each of 5 items (urticaria, fatigue, fever/chills, myalgia, and arthralgia/bone pain), which are clinical symptoms observed in patients with Schnitzler's syndrome, on a 5-point scale of 0 (no), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe) based on the physician's clinical examination to calculate the total score.

  2. Proportion of Patients Achieving Partial or Complete Clinical Response at all Time Points From 7 Days After the First Dose to 48 Weeks After the First Dose

    Time frame: Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

    Complete clinical response is defined as a PGA score of ≤ 5, with none of 5 items (urticaria, fatigue, fever and chills, myalgia, and arthralgia and bone pain) rated as > 1.

    Partial clinical response is defined as a PGA score of ≥ 6, with a decrease in PGA of ≥ 30% from baseline, or PGA score of ≤ 5, with any of 5 items (urticaria, fatigue, fever and chills, myalgia, and arthralgia and bone pain) rated as > 1.

    The PGA is a tool designed to evaluate each of 5 items (urticaria, fatigue, fever/chills, myalgia, and arthralgia/bone pain), which are clinical symptoms observed in patients with Schnitzler's syndrome, on a 5-point scale of 0 (no), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe) based on the physician's clinical examination to calculate the total score.

  3. Rate of Each Clinical Response

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

    The proportion of patients who achieve a clinical response:

    • Complete clinical response: PGA score of ≤ 5, with none of 5 items (urticaria, fatigue, fever and chills, myalgia, and arthralgia and bone pain) rated as > 1
    • Partial clinical response: PGA score of ≥ 6, with a decrease in PGA of ≥ 30% from baseline, or PGA score of ≤ 5, with any of 5 items (urticaria, fatigue, fever and chills, myalgia, and arthralgia and bone pain) rated as > 1
    • No clinical response: PGA score that increases, is stable, or decreases by < 30% as compared to baseline
    • Relapse or worsening of clinical symptoms: Increase in PGA score of ≥ 50% from baseline

    The PGA is a tool designed to evaluate each of 5 items, which are clinical symptoms observed in patients with Schnitzler's syndrome, on a 5-point scale of 0 (no), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe) based on the physician's clinical examination to calculate the total score.

  4. Mean PGA Score

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

    The PGA is a tool designed to evaluate each of 5 items (urticaria, fatigue, fever/chills, myalgia, and arthralgia/bone pain), which are clinical symptoms observed in patients with Schnitzler's syndrome, on a 5-point scale of 0 (no), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe) based on the physician's clinical examination to calculate the total score.

  5. Change From Baseline in PGA Score

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

    The PGA is a tool designed to evaluate each of 5 items (urticaria, fatigue, fever/chills, myalgia, and arthralgia/bone pain), which are clinical symptoms observed in patients with Schnitzler's syndrome, on a 5-point scale of 0 (no), 1 (minimal), 2 (mild), 3 (moderate), and 4 (severe) based on the physician's clinical examination to calculate the total score.

  6. Mean White Blood Cell Count

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  7. Change From Baseline in White Blood Cell Count

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  8. Mean Neutrophil Count

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  9. Change From Baseline in Neutrophil Count

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  10. Mean C-reactive Protein (CRP) Level

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  11. Change From Baseline in CRP Level

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  12. Mean Alkaline phosphatase (ALP) Level

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  13. Change From Baseline in ALP Level

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  14. Mean Serum Immunoglobulin (Ig) G and IgM Levels

    Time frame: Baseline, Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  15. Change From Baseline in Serum IgG and IgM Levels

    Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

  16. Rate of Physicians Global Assessment of Impression of Improvement

    Time frame: Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48

    The proportion of patients summarized by improvement status as assessed by the physician. Improvement assessment categories: markedly responded, responded, not responded, worsen, or indeterminate.

Secondary outcomes

  1. Number and Percentage of Patients With Adverse Reactions

    Time frame: From Baseline to Week 48

  2. Number and Percentage of Patients With Serious Adverse Events

    Time frame: From Baseline to Week 48

Study contacts

Contact information is provided by the study sponsor or research team.

Novartis Pharmaceuticals

CONTACT

[email protected]

+41613241111

Novartis Pharmaceuticals

CONTACT

Sponsors and collaborators

Lead sponsor

Novartis Pharmaceuticals

Industry

Registry information

Official study title

Specified Use-Results Study of ILARIS Solution for S.C. Injection (Schnitzler's Syndrome)

Important dates

Study start
2026
Primary completion
2032
Study completion
2032
First posted
May 20, 2026
Registry last updated
Jul 1, 2026

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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