Schnitzler SyndromeNCT07598422
A Study to Assess the Effectiveness and Safety of Canakinumab in Clinical Use in Patients With Schnitzler's Syndrome
An observational study of Schnitzler Syndrome, run by Novartis Pharmaceuticals at 5 sites in Japan.
Trial 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. Read more about this study
- Who is it for?
- Ages 0 years and older
- Conditions the study is for
- Schnitzler Syndrome
- How many people
- 10 participants
- Where
- 5 sitesJapan
- Study dates
- 13 May 2026to 25 Nov 2032
- Phase
- Observational
- Are there results?
- Not yet: the study is under way
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 0 years and older
- All sexes
- No healthy volunteers
- Child, Adult, Older adult
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.
Who may be able to join
- 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.
Who cannot join
- 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).
Trial locations and contacts
5 sites in 1 country. Contact the study team or a site near you to ask about taking part.
Novartis Pharmaceuticals contact
5 sites in 1 country
Japan5 sites, 5 recruiting
- Recruiting
Novartis Investigative Site
Kanoya, Kagoshima-ken, 8930024
- Recruiting
Novartis Investigative Site
Kobe, Hyōgo, 6500047
- Recruiting
Novartis Investigative Site
Osaka, 5458586
- Recruiting
Novartis Investigative Site
Ushiku, Ibaraki, 300-1231
- Recruiting
Novartis Investigative Site
Yokohama, Kanagawa, 2360004
About this study
Official title: Specified Use-Results Study of ILARIS Solution for S.C. Injection (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.
- Conditions
- Schnitzler Syndrome
- Keywords
- Schnitzler's syndrome, Canakinumab, ILARIS, Post-marketing surveillance, All-case surveillance
- Medical subject headings
- Schnitzler Syndrome
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
| Study group | Role | What they receive |
|---|---|---|
| Canakinumab Cohort | Patients diagnosed with Schnitzler's syndrome using canakinumab for the first time. |
What is being measured
The primary outcome is the main question the study is designed to answer.
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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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.
Measured: 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
Measured: 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
Measured: Baseline to Day 8, Weeks 8, 16, 24, 32, 40, and at treatment discontinuation, up to and including Week 48
Mean Neutrophil Count
Measured: 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
Measured: 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
Measured: 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
Measured: 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
Measured: 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
Measured: 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
Measured: 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
Measured: 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.
Measured: 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
Measured: From Baseline to Week 48
Number and Percentage of Patients With Serious Adverse Events
Measured: From Baseline to Week 48
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted20 May 2026
- Study start13 May 2026
- Primary completion25 Nov 2032 (estimated)
- Study completion25 Nov 2032 (estimated)
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Other trials for Schnitzler SyndromeStudies of the same condition, open and under way.
- More trials run by Novartis PharmaceuticalsThe sponsor's other registered studies.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Observational
- Observational model
- Cohort
- Time perspective
- Prospective
- Enrolment
- 10 participants (estimated)
Sponsor and investigators
- Lead sponsor
- Novartis Pharmaceuticals
- Sponsor type
- Industry (pharma, biotech, devices)
- Responsible party
- Sponsor
- Investigators
- Novartis Pharmaceuticals, Study director, Novartis Pharmaceuticals
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT07598422
- Sponsor's protocol ID
- CACZ885C21401
- Status last verified
- Sep 2026
- First submitted
- 14 May 2026
- Last updated
- 3 Sep 2026
- Results posted
- No
- Individual data sharing
- No
Study at a glance
From the registry record, last updated by the sponsor on 3 Sep 2026. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Specified Use-Results Study of ILARIS Solution for S.C. Injection (Schnitzler's Syndrome)
- ClinicalTrials.gov ID
- NCT07598422
- Acronym / sponsor ID
- CACZ885C21401
- Conditions
- Schnitzler Syndrome
- Keywords
- Schnitzler's syndrome, Canakinumab, ILARIS, Post-marketing surveillance, All-case surveillance
- Study type
- Observational
- Recruitment status
- Recruiting
- Enrolment
- 10 participants (estimated)
- Lead sponsor
- Novartis PharmaceuticalsIndustry (pharma, biotech, devices)
- Study start
- 13 May 2026 (actual)
- Primary completion
- 25 Nov 2032 (estimated)
- Study completion
- 25 Nov 2032 (estimated)
- First posted
- 20 May 2026 (actual)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
The aim of this study is to assess the effectiveness and safety of Ilaris (canakinumab) in clinical use in patients with Schnitzler's syndrome.
Study design
- Observational model
- Cohort
- Time perspective
- Prospective
- Number of arms
- 1
| Arm | Type | Receives |
|---|---|---|
| Canakinumab Cohort | – | –Patients diagnosed with Schnitzler's syndrome using canakinumab for the first time. |
Who may qualify
- Ages
- 0 years and older
- Sex
- All
- Healthy volunteers
- Not accepted
- Age groups
- Child, Adult, Older adult
- 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.
Key inclusion criteria (2 listed)
- 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.
Key exclusion criteria (4 listed)
- 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).
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Primary outcome
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.
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.
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.
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.
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.
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
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.
2 secondary outcomes
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
Results and safety
This study is still under way. Results are not available in the source record yet.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 3 Sep 2026.
- Individual participant dataSharing planned: No
Related trials for Schnitzler Syndrome
RecruitingObservational study
AutoInflammatory Disease Alliance Registry (AIDA)
Hereditary Autoinflammatory Diseases, Schnitzler Syndrome
Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part.
Novartis Pharmaceuticals contact
Novartis Pharmaceuticals study director
Novartis Pharmaceuticals
5 sites in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
An observational study of Schnitzler Syndrome, run by Novartis Pharmaceuticals at 5 sites in Japan.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 2 inclusion and 4 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: looking for participants now, as of the sponsor's last update on 3 Sep 2026. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 5 sites in Japan. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
This study is still under way. Results are not available in the source record yet.
How can a doctor refer a patient or contact the research team?
Contact Novartis Pharmaceuticals (+41613241111, novartis.email@novartis.com), or a site near the patient. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
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). The study lists 16 primary outcomes in all. It is what the study was designed to measure, not a finding.
Where can I read the original study information?
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 3 Sep 2026.