ß-SPECIFIC 4 Patients: Study of Pediatric EffiCacy and Safety wIth FIrst-line Use of Canakinumab
Condition: Systemic Juvenile Idiopathic Arthritis (SJIA)
MeSH terms: Arthritis; Arthritis, Juvenile
Intervention: ACZ885 150 mg (Canakinumab)
Intervention description: Active canakinumab in individual 2 mL glass vials, each containing 150 mg canakinumab liquid in vial.
Intervention MeSH terms: Antibodies, Monoclonal
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Allocation: Non-Randomized
Intervention model: Parallel Assignment
Intervention model description: A two-part open-label study that collected long-term efficacy, safety, and tolerability data from SJIA patients receiving canakinumab treatment who had inactive disease at the last visit in Study CACZ885G2301E1 (Cohort 1), and from SJIA patients who were canakinumab treatment-naïve and had active disease at the time of screening for this study (Cohort 2)
Masking: None (Open Label)
Masking description: No blinding was required in this open-label study
Enrollment: 182.0
Sex: All
Minimum age: 2 Years
Maximum age: 20 Years
Healthy volunteers: No
Keywords: Juvenile Rheumatoid arthritis (JRA) chronic; systemic inflammatory disorder; painful joints; inflammation of the synovial membrane; auto-immune rheumatoid disease; reactive rheumatoid arthritis; Systemic Juvenile Rheumatoid arthritis (SJRA)

Summary:

      The purpose of this study was to evaluate the efficacy observed with canakinumab dose
      reduction in a subgroup of patients in the extension study CACZ885G2301E1.
    

Eligibility criteria:

        Inclusion Criteria:

        Cohort 1:

        • Patients who are receiving canakinumab treatment (4 mg/kg every 4 weeks) for Systemic
        Juvenile Idiopathic Arthritis (SJIA) and have inactive disease at the last visit in Study
        CACZ885G2301E1

        Cohort 2:

          -  Confirmed diagnosis of SJIA as per International League Against Rheumatism (ILAR)
             definition that must have occurred at least 2 months prior to enrollment with an onset
             of disease < 16 years of age.

          -  Active SJIA defined as having 2 or more of the following:

          -  Documented spiking, intermittent fever (body temperature > 38°C) for at least 1 day
             within 1 week before first canakinumab dose;

          -  At least 2 joints with active arthritis

          -  C-reactive protein (CRP) > 30 mg/L (normal range < 10 mg/L)

          -  Rash due to SJIA

          -  Serositis

          -  Lymphadenopathy

          -  Hepatosplenomegaly

          -  Negative TB screen (QuantiFERON or, if required by local guidelines, Purified Protein
             Derivative).

        Exclusion Criteria:

          -  With active or recurrent bacterial, fungal or viral infection at the time of
             enrollment, including patients with evidence of Human Immunodeficiency Virus (HIV)
             infection, Hepatitis B and Hepatitis C infection.

          -  With 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.

          -  With neutropenia (absolute neutrophil count < 1500/mm3) at screening.