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

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