An Efficacy and Safety Study of GNR-086 (canakinumab Biosimilar) and Ilaris® in Patients with Adult-onset Still's Disease

Inclusion Criteria:

• Availability of written informed consent obtained from the patient before the startof any procedures related to the study.

• Male and female patients aged 18-75 years, inclusive, at the time of signing theinformed consent form.

• Patients with a documented diagnosis of adult Still's disease in accordance with theclassification criteria of Yamaguchi M. et al. (J. Rheumatology, 1992) and theduration of the disease is at least 2 months before signing the Informed ConsentForm.

• Disease activity ≥2.6 according to DAS28-ESR.

• No change in the dosing regimen of methotrexate (maximum 20 mg/m2/week) or otherimmunosuppressive agent for at least 6 weeks before randomization and/or no changein the dosing regimen of one nonsteroidal anti-inflammatory drug (NSAID) astreatment for adult Still's disease for at least 14 days before randomization and/orno change in the dosage regimen of the glucocorticosteroid drug for at least 7 daysbefore randomization.

• Negative result of intradermal test with tuberculosis allergen / IGRA test atscreening or within 6 months before screening. Patients with missing screening datacan only be included in the study if they undergo immunodiagnosis of tuberculosisinfection and the result is negative.

• Agreement to adhere to adequate methods of contraception throughout the study andfor 3 months after the end of canakinumab therapy.

Exclusion Criteria:

• Diagnosis of macrophage activation syndrome (MAS) within the last 3 months.

• History of hypersensitivity to the active substance or other components of the studyor reference drug.

• Acute infectious diseases within 14 days before randomization.

• Immunization with any live vaccine within 3 months before randomization.

• Concomitant diseases and conditions that, in the opinion of the Investigator and/orSponsor, jeopardize the safety of the patient during participation in the study, orwhich will influence the analysis of safety data.

• Blood donation or blood loss (450 ml of blood or more) less than 2 months before thestart of the study.

• Pregnancy or breastfeeding.

• History of tuberculosis (except for successfully treated primary tuberculosiscomplex no later than 6 months before randomization).

• Use of the following treatments before randomization: anakinra within 1 week beforerandomization; etanercept within 6 weeks before randomization; tocilizumab within 8weeks before randomization; sarilumab within 6 weeks before randomization;olokizumab for 8 weeks before randomization; adalimumab within 10 weeks beforerandomization; golimumab within 16 weeks before randomization; rituximab within 26weeks before randomization; leflunomide within 6 weeks before randomization;cyclosporine within 4 weeks before randomization; intravenous immunoglobulin within 8 weeks before randomization; growth hormone within 4 weeks before randomization;intra-articular, periarticular, intravenous, intramuscular administration ofglucocorticosteroids within 4 weeks before randomization; any other unapproved drugswithin 5 half-lives before randomization.

• Drug dependence on drugs and potent drugs and/or alcohol dependence.

• Positive test results for hepatitis B or C, HIV or syphilis.

• Unwillingness or inability to comply with the recommendations prescribed by thisprotocol.

• Identification during screening of other diseases/conditions not listed above that,in the opinion of the physician-researcher, prevent the inclusion of the patient inthe study.