RT Journal Article
T1 Efficacy and Safety of Janus Kinase Inhibitors in Type I Interferon-Mediated Monogenic Autoinflammatory Disorders: A Scoping Review
A1 Gómez-Arias, Pedro Jesús
A1 Gómez-García, Francisco
A1 Hernández-Parada, Jorge
A1 Montilla-López, Ana María
A1 Ruano, Juan
A1 Parra-Peralbo, Esmeralda
K1 CANDLE
K1 SAVI
K1 Familial chilblain lupus
K1 JAK inhibitors
K1 Autoinflammatory diseases
K1 Interferon pathway
K1 Ruxolitinib
K1 Tofacitinib
K1 Baricitinib
K1 Viremia
K1 Type I interferon
K1 Interferonopathies
K1 Inhibidores de las cinasas Janus
K1 Enfermedades autoinflamatorias hereditarias
K1 Interferón de tipo I
K1 Bases de datos bibliográficas
AB ImportanceType I interferon (IFN)-mediated monogenic autoinflammatory disorders (interferonopathies) are childhood-onset rare multisystemic diseases with limited treatment options. The Janus kinase (JAK) inhibitors are promising potential therapeutic candidates for immune-mediated chronic inflammatory skin diseases.ObjectiveTo review the use of JAK inhibitors to improve decision-making when treating interferonopathies with cutaneous manifestations.Evidence ReviewThe MEDLINE, EMBASE, CINAHL, Scopus, and Web of Science databases were searched for studies that used JAK protein inhibitors to treat IFN-related monogenic diseases with cutaneous manifestations in humans. The search results are reported using the scoping review approach.FindingsSeventeen open-label studies assessing the efficacy of ruxolitinib, baricitinib, or tofacitinib reported variable responses in patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) and related syndromes, stimulator of IFN genes [STING]-associated vasculopathy with onset in infancy (SAVI), familial chilblain lupus (FCh-L), gain-of-function mutations of STAT1 (GOF-STAT1), or Aicardi-Goutiéres syndrome. JAK inhibitors improved clinical and analytical parameters and decreased flare numbers, plasma inflammatory markers, and expression of IFN-stimulated genes. BK viremia and upper respiratory infections were the most frequent and severe adverse events. Significant heterogeneity in efficacy assessment methods and poor reporting of safety events were detected.Conclusions and RelevanceEvidence of the use of JAK inhibitors in patients with interpheronopathies is scarce and of low methodological quality. Future clinical trials should use validated scales and report drug safety in a more accurate way.
PB Springer Nature
SN 2193-8210
YR 2021
FD 2021-04-15
LK http://hdl.handle.net/10668/4440
UL http://hdl.handle.net/10668/4440
LA en
NO Gómez-Arias PJ, Gómez-García F, Hernández-Parada J, Montilla-López AM, Ruano J, Parra-Peralbo E. Efficacy and Safety of Janus Kinase Inhibitors in Type I Interferon-Mediated Monogenic Autoinflammatory Disorders: A Scoping Review. Dermatol Ther. 2021 Jun;11(3):733-750
DS RISalud
RD Apr 4, 2025