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

NCT07598422 | Not Yet Recruiting

• 1 other identifier: CACZ885C21401
• Study Type: observational
• Target: 5
• Locations: 0 countries
• Sites: N/A

Brief Summary

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

Conditions

Schnitzler Syndrome

Keywords

Schnitzler's syndrome; Canakinumab; ILARIS; Post-marketing surveillance; All-case surveillance

Outcome Measures

Primary Outcomes (16)

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

  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.

  Day 8 to Week 8

• 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

  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.

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

• Rate of Each Clinical Response

  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.

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

• Mean PGA Score

  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.

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

• Change From Baseline in PGA Score

  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.

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

• Mean White Blood Cell Count

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

• Change From Baseline in White Blood Cell Count

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

• Mean Neutrophil Count

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

• Change From Baseline in Neutrophil Count

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

• Mean C-reactive Protein (CRP) Level

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

• Change From Baseline in CRP Level

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

• Mean Alkaline phosphatase (ALP) Level

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

• Change From Baseline in ALP Level

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

• Mean Serum Immunoglobulin (Ig) G and IgM Levels

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

• Change From Baseline in Serum IgG and IgM Levels

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

• Rate of Physicians Global Assessment of Impression of Improvement

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

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

Secondary Outcomes (2)

• Number and Percentage of Patients With Adverse Reactions

  From Baseline to Week 48

• Number and Percentage of Patients With Serious Adverse Events

  From Baseline to Week 48

Study Arms (1)

Canakinumab Cohort

Patients diagnosed with Schnitzler's syndrome using canakinumab for the first time.

Eligibility Criteria

• Age: 0 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Schnitzler's syndrome patients receiving canakinumab for the first time for the indication of Schnitzler's syndrome as described in the package insert.

You may qualify if:

• \. 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.

You may not qualify if:

• 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).

Sponsors & Collaborators

• Novartis Pharmaceuticals: lead

MeSH Terms

Conditions

Schnitzler Syndrome

Condition Hierarchy (Ancestors)

Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Immunoproliferative Disorders; Immune System Diseases

Study Officials

• Novartis Pharmaceuticals

  Novartis Pharmaceuticals

  STUDY DIRECTOR

Central Study Contacts

Novartis Pharmaceuticals

CONTACT

+41613241111; novartis.email@novartis.com

Novartis Pharmaceuticals

CONTACT

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: INDUSTRY
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: May 14, 2026
• First Posted: May 20, 2026
• Study Start (Estimated): May 31, 2026
• Primary Completion (Estimated): June 30, 2032
• Study Completion (Estimated): June 30, 2032
• Last Updated: May 20, 2026

Record last verified: 2026-05

Data Sharing

• IPD Sharing: Will not share