Schnitzler syndrome is an acquired autoinflammatory syndrome characterized by urticaria and monoclonal gammopathy, accompanied by intermittent fever, arthralgia or arthritis, bone pain and lymphadenopathy. This chronic disease severely impedes the quality of life of the patients.
Conditions
Interventions
Trade Name: Ilaris
Product Name: canakinumab
Pharmaceutical Form: Powder for suspension for injection
INN or Proposed INN: CANAKINUMAB
CAS Number: 914613-48-2
Concentration unit: mg milligram(s)
Conce
Sponsors
Radboud University Nijmegen Medical Centre
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1. Patients with a diagnosis of Schnitzler syndrome as per criteria (see Table 1). 2. Patients that have been / are treated with Anakinra must have demonstrated a partial or complete clinical response with an associated normalization of their biomarkers of inflammation (CRP). 3. Male and female patients at least 18 years of age at the time of the screening visit. 4. Patient’s informed consent. 5. Negative QuantiFERON test or negative Purified Protein Derivative (PPD) test (=65 years) yes F.1.3.1 Number of subjects for this age range
Exclusion criteria
Exclusion criteria: 1. Pregnant or nursing (lactating) women 2. History of being immunocompromised, including a positive HIV at screening (ELISA and Western blot). 3. Serologic evidence of hepatitis B or C infection 4. Live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose 5. History of significant medical conditions, which in the Investigator’s opinion would exclude the patient from participating in this trial 6. History of recurrent and/or evidence of active bacterial, fungal, or viral infection(s) 7. Use of the following therapies: • Anakinra within 24 hours prior to 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 8. History of hypersensitivity to any of the study drugs or to drugs of similar chemical classes
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Main Objective: Primary: To assess if canakinumab can induce complete or almost complete response in patients with symptomatic Schnitzler syndrome at Day 14.;Secondary Objective: Secondary: 1. To assess if canakinumab can induce complete or almost complete response in patients with symptomatic Schnitzler syndrome at Day 3 and Day 7 2. To assess if canakinumab can induce clinical remission at Day 3, Day 7 and Day 14 3. To assess if canakinumab can prevent disease relapse in patients who demonstrated complete remission at Day 14 4. To assess the change in CRP and SAA during the treatment and follow-up periods 5. To assess the change in physician and patient global assessment of disease activity during the treatment and follow-up periods 6. To assess the time to relapse after the last canakinumab dose 7. To evaluate the safety and tolerability of canakinumab in the treatment of patients with Schnitzler syndrome. 8. To assess PK/PD properties of canakinumab in patients with Schnitzler syndrome ;Primary end point(s): Percentage of patients with a complete or almost complete response in patients with symptomatic Schnitzler syndrome at Day 14 after canakinumab administration. Secondary: 1. To assess if canakinumab can induce complete or almost complete response in patients with symptomatic Schnitzler syndrome at Day 3 and Day 7 2. To assess if canakinumab can induce clinical remission at Day 3, Day 7 and Day 14 3. To assess if canakinumab can prevent disease relapse in patients who demonstrated complete remission at Day 14 4. To assess the change in CRP and SAA during the treatment and follow-up periods 5. To assess the change in physician and patient global assessment of disease activity during the treatment and follow-up periods 6. To assess the time to relapse after the last canakinumab dose 7. To evaluate the safety and tolerability of canakinumab in the treatment of patients with Schnitzler syndrome. 8. To assess PK/PD properties of canakinumab in patients with | — |
Countries
Netherlands
Outcome results
None listed