Efficacy and Safety of Janus Kinase Inhibitors in Type I Interferon-Mediated Monogenic Autoinflammatory Disorders: A Scoping Review

Type I interferon (IFN)-mediated monogenic autoinflammatory disorders (interferonopathies) are childhood-onset rare multisystemic diseases with limited treatment options. The Janus kinase (JAK) inhibitors promising potential therapeutic candidates for immune-mediated chronic inflammatory skin diseases. To review the use of JAK to improve decision-making when treating interferonopathies cutaneous manifestations. MEDLINE, EMBASE, CINAHL, Scopus, and Web Science databases were searched studies that used protein treat IFN-related manifestations in humans. search results reported using scoping approach. Seventeen open-label assessing efficacy ruxolitinib, baricitinib, or tofacitinib variable responses patients atypical neutrophilic dermatosis lipodystrophy elevated temperature (CANDLE) related syndromes, stimulator IFN genes [STING]-associated vasculopathy onset infancy (SAVI), familial chilblain lupus (FCh-L), gain-of-function mutations STAT1 (GOF-STAT1), Aicardi-Goutieres syndrome. improved clinical analytical parameters decreased flare numbers, plasma markers, expression IFN-stimulated genes. BK viremia upper respiratory infections most frequent severe adverse events. Significant heterogeneity assessment methods poor reporting safety events detected. Evidence interpheronopathies is scarce low methodological quality. Future trials should validated scales report drug in a more accurate way.