A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD)
Condition: Still's Disease, Adult-Onset; Still's Disease, Juvenile-Onset
MeSH terms: Arthritis, Juvenile; Still's Disease, Adult-Onset
Intervention: anakinra; Placebo
Intervention description: sub cutaneous injection; sub cutaneous injection
Intervention MeSH terms: Interleukin 1 Receptor Antagonist Protein
Phase: Phase 3
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)
Enrollment: 13.0
Sex: All
Healthy volunteers: No
Keywords: Interleukin 1 receptor antagonist; IL-1 receptor antagonist; Kineret; anakinra; Adult-Onset Still's Disease; Systemic Juvenile Idiopathic Arthritis

Summary:

      The aim of this study is to demonstrate the efficacy and to evaluate the safety,
      pharmacokinetics (PK) and immunogenicity of anakinra in patients with newly diagnosed Still's
      disease, including SJIA (Systemic juvenile idiopathic arthritis) and AOSD (Adult-onset
      Still's disease).
    

Eligibility criteria:

        Inclusion Criteria:

          1. Signed informed consent.

          2. Male and female patients with a body weight ≥ 10 kg.

          3. Diagnosis of Still's disease.

          4. If currently on glucocorticoid treatment, a stable dose for at least 1 week prior to
             randomization.

          5. If currently on methotrexate treatment, a stable dose for at least 8 weeks prior to
             randomization.

          6. Active disease.

          7. Female patients of childbearing potential must use an effective method of
             contraception during the study (abstinence being a possible option) as well as present
             a negative pregnancy test prior to randomization.

          8. Negative interferon-gamma release assay or Purified protein derivative ( PPD) test
             within 2 months prior to randomization. If not available, a test should be performed
             at day of randomization.

        Exclusion Criteria:

          1. Diagnosis of Still's disease more than 6 months prior to randomization.

          2. Previous randomization into this study.

          3. Participation in another concurrent clinical interventional study within 30 days of
             randomization.

          4. Treatment with an investigational drug within 5 half-lives prior to randomization.

          5. Previous or current treatment with anakinra, canakinumab or any other IL-1 inhibitor.

          6. Use of the following therapies prior to randomization:

               -  Narcotic analgesics within 24 hours prior to randomization.

               -  Dapsone or etanercept within 3 weeks prior to randomization.

               -  Intraarticular, intramuscular or intravenous administration of glucocorticoids or
                  intravenous immunoglobulin (Ig) within 4 weeks prior to randomization.

               -  Intravenous Ig with proven Still's disease modifying effect, leflunomide,
                  infliximab or adalimumab within 8 weeks prior to randomization.

               -  Thalidomide, cyclosporine, mycophenolate mofetil, 6-mercaptopurine, azathioprine,
                  cyclophosphamide, chlorambucil or any other immunosuppressant within 12 weeks
                  prior to randomization.

               -  Tocilizumab within 12 weeks prior to randomization or any other immunomodulatory
                  medication within 4 half-lives prior to randomization

               -  Rituximab within 26 weeks prior to randomization.

          7. Live vaccines within 1 month prior to randomization.

          8. Known presence or suspicion of active, chronic or recurrent bacterial, fungal or viral
             infections, including tuberculosis, HIV infection or hepatitis B or C infection.

          9. Clinical evidence of liver disease or liver injury.

         10. Presence of severe renal function impairment.

         11. Presence of neutropenia.

         12. Presence or suspicion of MAS at baseline.

         13. A diagnosis of MAS within the last 2 months prior to randomization.

         14. History of malignancy within 5 years.

         15. Known hypersensitivity to E coli-derived proteins, or any components of Kineret®
             (anakinra).

         16. Pregnant or lactating women.

         17. Foreseeable inability to cooperate with given instructions or study procedures.

         18. Presence of any medical or psychological condition or laboratory result that in the
             opinion of the investigator can interfere with the patient's ability to comply with
             the protocol requirements or makes the patient not appropriate for inclusion to the
             study and treatment with IMP.