An observational study in Schnitzler Syndrome, sponsored by Novartis Pharmaceuticals. Recruiting at 5 sites in Japan. Open to participants aged 0 Years and older. Per ClinicalTrials.gov, last updated 2026-09-03.
Sponsored by Novartis Pharmaceuticals · Observational
The aim of this study is to assess the effectiveness and safety of Ilaris (canakinumab) in clinical use in patients with Schnitzler's syndrome.
Novartis Pharmaceuticals is the lead sponsor of 2,673 studies on the registry; 228 are open to participants now.
Of its 576 completed or terminated interventional studies of FDA-regulated products, 431 (75%) have results posted.
Counted across the registry records on this site, refreshed daily.
Schnitzler's syndrome patients receiving canakinumab for the first time for the indication of Schnitzler's syndrome as described in the package insert.
1. 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
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 diagnosed with Schnitzler's syndrome using canakinumab for the first time.
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.
Time frame: 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.
Time frame: 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.
Time frame: 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.
Time frame: 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.
Time frame: 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
Time frame: 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
Time frame: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48
Mean Neutrophil Count
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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
Time frame: 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.
Time frame: Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48
Number and Percentage of Patients With Adverse Reactions
Time frame: From Baseline to Week 48
Number and Percentage of Patients With Serious Adverse Events
Time frame: From Baseline to Week 48
Plan to share: No
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Schnitzler Syndrome
Novartis Pharmaceuticals