Safety, Efficacy, Pharmacokinetics, Pharmacodynamics of ACZ885 in Patients With NALP3 Mutations and Clinical Symptoms

Inclusion Criteria: * Patients aged 4 to 75 years (inclusive) * Body weight ≥ 12 kg and \< 100 kg. * Females of child-bearing potential must have a negative pregnancy test. Additional birth control details to be provided at screening. * Documented molecular diagnosis of NALP3 mutations and clinical symptoms that are either untreated or insufficiently treated and require medical intervention. * Patients under anakinra therapy or any other IL-1 blocking therapy, whose clinical symptoms improved under treatment and are willing to discontinue that therapy until a relapse becomes evident. * Patients with a very severe characteristics requiring oral prednisone are eligible if the dose is stable (≤ 0.4 mg/kg/day or ≤ 20 mg/day, whichever is lower) for at least 1 week prior to the screening visit. Steroid therapy may be tapered during treatment with ACZ885 at the discretion of the investigator. * Parents' or legal guardian's written informed consent (patient's informed consent for ≥ 18 years of age) and child's assent, if appropriate, are required prior to study participation. Exclusion Criteria: * Participation in any clinical trial investigation (except trials with anakinra) within 4 weeks prior to dosing or longer per local regulation * Antiinflammatory therapy with colchicine, chlorambucil, dapsone, azathioprine, mycophenolate mofetil, within 3 weeks prior to dosing. Therapeutic antibodies (e.g. anti-TNF-alpha antibodies) must be discontinued at least 60 days before dosing. * Donation or loss of 400 mL or more of blood within 8 weeks prior to dosing. * A past personal or close family medical history of clinically significant ECG abnormalities or prolonged QT-interval syndrome. * History of * Immunocompromise, including a positive HIV result. * Positive Hepatitis B surface antigen or Hepatitis C test result. * Drug or alcohol abuse within the 12 months prior to dosing. * Tuberculosis. * Renal transplant. * Evidence of lymphoma. * Active medical condition preventing participation in the study such as infection, poorly controlled diabetes etc. * No live vaccinations within 3 months prior to the start of the trial, during the trial, and up to 3 months following the last dose.