A Study of Baricitinib (LY3009104) in Adult and Pediatric Japanese Participants With NNS/CANDLE, SAVI, and AGS

A Phase 2/3, Multicenter, Open-Label Study to Evaluate the Efficacy and Safety of Baricitinib in Adult and Pediatric Japanese Patients With NNS/CANDLE, SAVI, and AGS

The main purpose of this study is to evaluate the efficacy and safety of baricitinib in adult and pediatric Japanese participants with Nakajo-Nishimura Syndrome/chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (NNS/CANDLE), STING-associated vasculopathy with onset during infancy (SAVI), and Aicardi-Goutières Syndrome (AGS).

Inclusion Criteria:

• Have systemic signs and symptoms of inflammation as manifested NNS/CANDLE, SAVI, AGS
• Have been diagnosed with genetic diagnosis
• Men must agree to use a reliable method of birth control during the study
• Women not of child-bearing potential or nonbreastfeeding
• Women must agree to use birth control or remain abstinent during the study and for at least 12 weeks after stopping treatment
• NNS/CANDLE and SAVI patients who are ≥17.5 months of age
• AGS patients who are ≥6 months of age
• Are ≥ 5kg in body weight

Exclusion Criteria:

• Have received immunosuppressive biologic agent/monoclonal antibody/oral JAK inhibitor/OAT3 inhibitor and cannot discontinue prior to investigational product initiation. Note: A washout period is required to each drug.
• Have diagnosis of current active tuberculosis (TB) or, latent TB who did not receive appropriate treatment.
• Have had a serious infection within 12 weeks prior to screening.
• Have a history of lymphoproliferative disease
• Have any history of venous thromboembolic event (VTE) (deep vein thrombosis [DVT]/pulmonary embolism [PE]) prior to screening.
• Have had any major surgery within 8 weeks prior to screening.
• Have previously been enrolled in any other study investigating baricitinib.