Efficacy and safety of canakinumab in Schnitzler syndrome

Recruitment & Eligibility

Status: Recruiting

Sex: Not specified

Target Recruitment: 10

Inclusion Criteria

1. Patients with a diagnosis of Schnitzler syndrome as fulfilling the criteria (see table 1).
2. Patients with Schnitzler syndrome who have been treated with the biological agent Anakinra must have demonstrated a partial or complete clinical response with an associated decrease in their biomarkers of inflammation (CRP and SAA) below 10mg/L.
3. Active Schnitzler syndrome (if applicable after stop of current treatment) at time of start of study treatment.
4. Male and female patients at least 18 years of age at the time of the screening visit.
5. Patient*s informed consent prior to the study
6. Negative QuantiFERON test or negative Purified Protein Derivative (PPD) test (< 5 mm induration) at screening or within 1 month prior to the screening visit, according to the national guidelines. Patients with a positive PPD test (>= 5 mm induration) at screening may be enrolled only if they have either a negative chest x-ray or a negative QuantiFERON test (QFT-TB G In-Tube.
7. Adequate contraception in premenopausal women

Exclusion Criteria

1. Pregnant or nursing (lactating) women
2. History of being immunocompromised, including a positive HIV at screening (ELISA and Western blot).
3. Live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose
4. History of significant medical conditions, which in the Investigator*s opinion would exclude the patient from participating in this trial
5. History of recurrent and/or evidence of active bacterial, fungal, or viral infection(s)
6. Use of any other medication to control the symptoms of active Schnitzler syndrome.
7. Corticosteroids > 0.1 mg/kg/day in the 1 week prior to the baseline visit
8. Use of the following therapies:
• Anakinra within 24 hours prior to Baseline visit
• Rilonacept within 1 week prior to Baseline visit
• Toclizumab within 3 weeks prior to Baseline visit
• Etanercept within 4 weeks prior to Baseline visit
• Adalimumab within 8 weeks prior to the Baseline visit
• Infliximab within 12 weeks prior to the Baseline visit
• Rituximab within 26 weeks prior to the Baseline visit
• Leflunomide within 4 weeks prior to the Baseline visit. Documentation of a completion of a full cholestyramine elimination treatment after most recent leflunomide use will be required.
• Thalidomide within 4 weeks prior to the Baseline visit
• Cyclosporine within 4 weeks prior to the Baseline visit
• Intravenous immunoglobulin (i.v. Ig) within 8 weeks prior to the Baseline visit
• 6-Merceptopurine, azathioprine, cyclophosphamide, or chlorambucil within 12 weeks prior to the Baseline visit
• Dapsone, mycophenolate mofetil within 3 weeks prior to the Baseline visit
• Growth hormone within 4 weeks prior to the Baseline visit
• Corticosteroids (oral prednisone (or equivalent)) > 1.0 mg/kg/day (or greater than the maximum of 60 mg/day for children over 60 kg) within 3 days prior to the Baseline visit
• Intra-articular, peri-articular or intramuscular corticosteroid injections within 4 weeks prior to the Baseline visit
• Any other investigational biologics within 8 weeks prior to the Baseline visit
• Any other investigational drugs, other than investigational biologic treatment, within 30 days (or 3 months for investigational monoclonal antibodies) or 5 half-lives prior to the Baseline visit, whichever is longer
9. History of hypersensitivity to any of the study drugs or to drugs of similar chemical classes
10. Severe co-morbidity (as judged by investigator).

Study & Design

Study Type: Interventional

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>Complete or clinical remission at Day 14.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>1. Complete or clinical remission at Day 3 and Day 7<br /><br>2. The prevention of disease relapse in patients who demonstrated complete<br /><br>remission at Day 14<br /><br>3. The change in biomarkers (CRP and SAA) and clinical parameters (physician<br /><br>and patient global assessment of disease activity) during the treatment and<br /><br>follow-up periods<br /><br>4. Time to relapse after the last canakinumab dose<br /><br>5. Safety and tolerability as well as PK/PD properties of canakinumab in the<br /><br>treatment of patients with Schnitzler syndrome.<br /><br>Exploratory:<br /><br>1. Changes in patient quality of life by using: Medical Outcome Short Form (36)<br /><br>Health Survey (SF-36®).<br /><br>2. Optimal canakinumab dose and frequency in patients with Schnitzler syndrome</p><br>