A Study to Evaluate Efficacy and Safety of Anakinra in the Treatment of Still's Disease (SJIA and AOSD)

The study consists of a 12-week, randomized, double-blind, placebo controlled period with two
dose levels of anakinra and a 4-week safety follow-up after last dose of investigational
medicinal product (IMP). The primary endpoint will be evaluated at Week 2. Sustained efficacy
and time to early termination will be evaluated during the full study period.

A screening visit is optional and may be done to identify patients that could be suitable for
the study. During the study 6 visits and 2 telephone contacts are scheduled i.e., Day 1
(baseline visit), Day 4Tel, Week 1, Week 2, Week 4, Week 8, Week 12 and Week 16Tel (End of
Study).

Patients will be randomly assigned to study drug, after they meet all of the inclusion
criteria and none of the exclusion criteria. Patients will receive treatment for 12 weeks,
either anakinra or placebo. Patients will be randomized to anakinra in a dose of either 2 or
4 mg/kg/day, with a maximum dose of 100 or 200 mg once daily, respectively. Patients will be
randomized to placebo with corresponding volumes for each of the two anakinra dose levels.

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.

- Tocilizumab, dapsone or mycophenolate mofetil within 3 weeks prior to
randomization.

- Etanercept, leflunomide, thalidomide, or cyclosporine or intraarticular,
intramuscular or intravenous administration of glucocorticoids within 4 weeks
prior to randomization.

- Intravenous immunoglobulin (i.v. Ig) or adalimumab within 8 weeks prior to
randomization.

- Infliximab, 6-mercaptopurine, azathioprine, cyclophosphamide or chlorambucil
within 12 weeks 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. Current signs and symptoms of MAS as judged by the investigator.

13. A diagnosis of MAS within the last 2 months prior to randomization.

14. History of malignancy.

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.