Background
inflammation, CANDLE, AGS, monogenic disorders, vasculopathy, lipodystrophy
PROLIFERATION, INFLAMMATION, LIPODYSTROPHY, NEUTROPHILIC DERMATOSIS
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE), stimulator of interferon genes (STING)-associated vasculopathy with onset during infancy (SAVI), and Aicardi-Goutières syndrome (AGS) are Mendelian autoinflammatory interferonopathies characterized by early-onset systemic and organ-specific inflammation and a prominent interferon (IFN) response gene signature (IGS) [CANDLE, SAVI, and AGS are monogenic disorders caused by pathogenic genetic defects [Considering the dysregulation of IFNs in CANDLE, SAVI, and AGS, an anti-IFN approach could be a possible therapeutic option. Moreover, due to the presence of a high IGS, patients with CANDLE and SAVI respond poorly to disease-modifying antirheumatic drugs and interleukin-1-blocking agents [Baricitinib is a selective JAK1/2 inhibitor that reduces the phosphorylation and activation of STATs, consequently modulating inflammation, cellular activation, and proliferation of key immune cells [This study evaluates the efficacy and safety of baricitinib in pediatric and adult Japanese patients with NNS/CANDLE, SAVI, and AGS. The safety and tolerability data from this study are intended to establish an understanding of the benefit/risk relationship of baricitinib in patients with NNS/CANDLE, SAVI, and AGS.