An Open-label Extension Study of Canakinumab in Patients With Systemic Juvenile Idiopathic Arthritis and Active Systemic Manifestations Manifestations and Response Characterization Study in Canakinumab Treatment-naïve Patients With Active SJIA With and Without Fever. (β-SPECIFIC 3)

• Assess the long-term safety, tolerability and immunogenicity of canakinumab. [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: Yes ]
• Maintenance of at least an adapted ACR pediatric 30. [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: No ]
• Primary Outcome Measure #2: Measure: Assess efficacy of canakinumab treatment based on adapted ACR pediatric 30 criteria in patients who report previous anakinra, tocilizumab or other biologic treatment [ Time Frame: Day 1 to 728 ] [ Designated as safety issue: No ]

• The number of patients that were able to taper steroid as per protocol. [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: No ]
• The number of patients who reached steroid free regimen. [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: No ]
• The number of patients who were able to reduce the canakinumab dose to 2 mg/kg/4 week [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: No ]
• The percentage of patients who will meet the definition of inactive disease on medication and possible clinical remission on medication [ Time Frame: Days 1 to 728 ] [ Designated as safety issue: No ]

This open-label extension study will permit patients with Systemic Juvenile Idiopathic Arthritis (SJIA) who previously were responsive to treatment with canakinumab and canakinumab treatment-naïve patients with active SJIA with and without fever to be retreated with 4 mg/kg s.c. every 4 weeks and assessed for continued efficacy and safety until discontinuation or when study CACZ885G2402 is in place at their study center or around March 2013, whichever occurs first. Patients who are steroid-free will be able to taper their canakinumab dose to 2 mg/kg s.c. every 4 weeks.

Inclusion criteria:

• Patients from study CACZ885G2305 or CACZ885G2301 who achieved an adapted ACR pediatric 30 response 15 days after their initial dose of canakinumab but clinically deteriorated afterwards or a minimum ACR Pediatric 30 response was not maintained after Day 15 and intervention is deemed necessary by the investigator, or Patients in study CACZ885G2301 who are not eligible to enter Part II (withdrawal part) because they were not able to meet the corticosteroid entry criteria , or Responder patients in Part I or Part II who had not flared when CACZ885G2301 was stopped, or CACZ885G2301 patients who were responders in Part I but experienced a flare in Part II.

• Treatment-naïve patients need to meet the following criteria:

  • Confirmed diagnosis of systemic juvenile idiopathic arthritis as per ILAR definition that must have occurred at least 2 months prior to enrollment with onset of disease < 16 years of age
  • Male and female patients aged ≥ 2 to < 20 years of age

  • Active disease at the time of enrollment 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
    • AND C-reactive protein (CRP) > 30 mg/L (normal range < 10 mg/L) Rash Serositis Lymphadenopathy Hepatosplenomegaly
  • Naïve to canakinumab

Other protocol-defined inclusion criteria may apply

Exclusion criteria:

• History of allergy or hypersensitivity to study drug
• With active or recurrent bacterial, fungal or viral infections at time of enrollment

Other protocol inclusion/exclusion criteria may apply

• Pediatric Rheumatology International Trials Organization
• Pediatric Rheumatology Collaborative Study Group