An Open-label, Multi-arm, Non-comparative Safety and Tolerability Study of Canakinumab (ACZ885) in Patients With Active Systemic Juvenile Idiopathic Arthritis (SJIA)

Key inclusion criteria: Cohort 1: 1\. All patients currently enrolled in study CACZ885G2301E1, including patients who discontinued canakinumab therapy for inactive disease in CACZ885G2301E1 as per physician discretion and who are now currently in a flare and require canakinumab therapy again Cohort 2: 1. Male and female patients aged ≥ 2 to \< 20 years at the time of the screening visit 2. Confirmed diagnosis of SJIA as per ILAR definition that must have occurred at least 2 months prior to enrollment with an onset of disease \< 16 years of age: • Arthritis in one or more joints, with or preceded by fever of at least 2 weeks duration that is documented to be daily/quotidian for at least 3 days and accompanied by one or more of the following: * Evanescent non-fixed erythematous rash, * Generalized lymph node enlargement, * Hepatomegaly and/ or splenomegaly, * Serositis 3. Active systemic disease at the time of baseline visit defined as having 2 or more of the following: * Documented spiking, intermittent fever (body temperature \> 38°C) for at least 1 day during the screening period and within 1 week before first canakinumab dose, * At least 2 joints with active arthritis (using ACR definition of active joint), * C-reactive protein (CRP) \> 30 mg/L (normal range \< 10 mg/L), * Rash, * Serositis, * Lymphadenopathy, * Hepatosplenomegaly 4. Patient's willingness to discontinue anakinra, rilonacept, tocilizumab or other experimental drug under close monitoring 5. Patients who are scheduled to receive an immunization, according to their local vaccination guidelines, with an inactivated vaccine and willing to participate in the assessment schedule for vaccinated patients Key exclusion criteria: Cohort 1 and Cohort 2: 1. Active or recurrent bacterial, fungal or viral infection at the time of enrollment 2. Underlying metabolic, renal, hepatic, infectious or gastrointestinal conditions which in the opinion of the investigator immunocompromises the patient and/ or places the patient at unacceptable risk for participation in an immunomodulatory therapy. 3. History of malignancy of any organ system (other than localized basal cell carcinoma of the skin), treated or untreated, within the past 5 years, regardless of whether there is evidence of local recurrence or metastases. 4. Live vaccinations within 3 months prior to the start of the study. Cohort 2: The following additional key exclusion criteria apply for Cohort 2. 1. Presence of moderate to severe impaired renal function 2. Clinical evidence of liver disease or liver injury as indicated by abnormal liver function tests at screening 3. History/evidence of macrophage activation syndrome within the previous 6 months Other protocol-defined inclusion/exclusion criteria may apply.