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.
Condition: Systemic Juvenile Idiopathic Arthritis
MeSH terms: Arthritis; Arthritis, Juvenile
Intervention: Canakinumab
Intervention description: Canakinumab
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 270.0
Sex: All
Minimum age: 2 Years
Maximum age: 19 Years
Healthy volunteers: No
Keywords: Flare; arthritis; IL-1beta antagonist; systemic juvenile idiopathic arthritis; CHAQ; steroids; ACR pediatric response; Systemic juvenile idiopathic arthritis with active flare

Summary:

      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.
    

Eligibility criteria:

        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