Efficacy and Safety of Canakinumab in Schnitzler Syndrome

Inclusion Criteria: * Patients with a diagnosis of Schnitzler syndrome as per criteria (ref 1). * 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). * Male and female patients at least 18 years of age at the time of the screening visit. * Patient's informed consent. * 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). * Adequate contraception in premenopausal females Exclusion Criteria: * Pregnant or nursing (lactating) women * History of being immunocompromised, including a positive HIV at screening (ELISA and Western blot). * Serologic evidence of hepatitis B or C infection * Live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose * History of significant medical conditions, which in the Investigator's opinion would exclude the patient from participating in this trial * History of recurrent and/or evidence of active bacterial, fungal, or viral infection(s) * Use of the following therapies: * Anakinra within 24 hours prior to Baseline visit XML File Identifier : tl8ybe8lI1o6DeawQocCBa8TF/w= * 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 * History of hypersensitivity to any of the study drugs or to drugs of similar chemical classes