{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T23:50:49Z","timestamp":1788306649429,"version":"build-2803163510"},"reference-count":35,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2021,4,15]],"date-time":"2021-04-15T00:00:00Z","timestamp":1618444800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"},{"start":{"date-parts":[[2021,4,15]],"date-time":"2021-04-15T00:00:00Z","timestamp":1618444800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"funder":[{"DOI":"10.13039\/501100010566","name":"Consejer\u00eda de Salud, Junta de Andaluc\u00eda","doi-asserted-by":"publisher","award":["PIN-0316-2017"],"award-info":[{"award-number":["PIN-0316-2017"]}],"id":[{"id":"10.13039\/501100010566","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Dermatol Ther (Heidelb)"],"published-print":{"date-parts":[[2021,6]]},"DOI":"10.1007\/s13555-021-00517-9","type":"journal-article","created":{"date-parts":[[2021,4,15]],"date-time":"2021-04-15T15:23:00Z","timestamp":1618500180000},"page":"733-750","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["Efficacy and Safety of Janus Kinase Inhibitors in Type I Interferon-Mediated Monogenic Autoinflammatory Disorders: A Scoping Review"],"prefix":"10.1007","volume":"11","author":[{"given":"Pedro Jes\u00fas","family":"G\u00f3mez-Arias","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco","family":"G\u00f3mez-Garc\u00eda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge","family":"Hern\u00e1ndez-Parada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Mar\u00eda","family":"Montilla-L\u00f3pez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Ruano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esmeralda","family":"Parra-Peralbo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,4,15]]},"reference":[{"issue":"68","key":"517_CR1","doi-asserted-by":"publisher","first-page":"297","DOI":"10.1146\/annurev-med-050715-104506","volume":"14","author":"MA Lee-Kirsch","year":"2017","unstructured":"Lee-Kirsch MA. The type i interferonopathies. Annu Rev Med. 2017;14(68):297\u2013315.","journal-title":"Annu Rev Med"},{"key":"517_CR2","doi-asserted-by":"crossref","unstructured":"Fr\u00e9mond M-L, Crow YJ. Mendelian disorders of immunity related to an upregulation of type I interferon. In: Sullivan K, Stiehm ER, editors. Stiehm\u2019s immune deficiencies. Amsterdam: Elsevier; 2020. p. 751\u201372.","DOI":"10.1016\/B978-0-12-816768-7.00032-6"},{"issue":"8","key":"517_CR3","doi-asserted-by":"publisher","first-page":"1758","DOI":"10.3389\/fimmu.2017.01758","volume":"11","author":"Y Nan","year":"2017","unstructured":"Nan Y, Wu C, Zhang YJ. Interplay between Janus kinase\/signal transducer and activator of transcription signaling activated by type I interferons and viral antagonism. Front Immunol. 2017;11(8):1758.","journal-title":"Front Immunol"},{"issue":"12","key":"517_CR4","doi-asserted-by":"publisher","first-page":"2527","DOI":"10.1084\/jem.20161596","volume":"213","author":"MP Rodero","year":"2016","unstructured":"Rodero MP, Crow YJ. Type I interferon-mediated monogenic autoinflammation: the type I interferonopathies, a conceptual overview. J Exp Med. 2016;213(12):2527\u201338.","journal-title":"J Exp Med"},{"issue":"6","key":"517_CR5","doi-asserted-by":"publisher","first-page":"507","DOI":"10.1056\/NEJMoa1312625","volume":"371","author":"Y Liu","year":"2014","unstructured":"Liu Y, Jesus AA, Marrero B Y, et al. Activated STING in a vascular and pulmonary syndrome. N Engl J Med. 2014;371(6):507\u201318.","journal-title":"N Engl J Med."},{"key":"517_CR6","doi-asserted-by":"publisher","first-page":"927","DOI":"10.3389\/fimmu.2017.00927","volume":"8","author":"A Torrelo","year":"2017","unstructured":"Torrelo A. CANDLE syndrome as a paradigm of proteasome-related autoinflammation. Front Immunol. 2017;8:927.","journal-title":"Front Immunol"},{"issue":"3","key":"517_CR7","doi-asserted-by":"publisher","first-page":"895","DOI":"10.1002\/art.33368","volume":"64","author":"Y Liu","year":"2012","unstructured":"Liu Y, Ramot Y, Torrelo A, et al. Mutations in proteasome subunit \u03b2 type 8 cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature with evidence of genetic and phenotypic heterogeneity. Arthritis Rheum. 2012;64(3):895\u2013907.","journal-title":"Arthritis Rheum"},{"issue":"7","key":"517_CR8","doi-asserted-by":"publisher","first-page":"517","DOI":"10.1097\/DAD.0000000000000340","volume":"37","author":"A Torrelo","year":"2015","unstructured":"Torrelo A, Colmenero I, Requena L, et al. Histologic and immunohistochemical features of the skin lesions in CANDLE syndrome. Am J Dermatopathol. 2015;37(7):517\u201322.","journal-title":"Am J Dermatopathol"},{"key":"517_CR9","doi-asserted-by":"publisher","first-page":"1535","DOI":"10.3389\/fimmu.2018.01535","volume":"9","author":"RG Saldanha","year":"2018","unstructured":"Saldanha RG, Balka KR, Davidson S, et al. A mutation outside the dimerization domain causing atypical STING-associated vasculopathy with onset in infancy. Front Immunol. 2018;9:1535.","journal-title":"Front Immunol."},{"issue":"3","key":"517_CR10","doi-asserted-by":"publisher","first-page":"431","DOI":"10.1136\/annrheumdis-2018-214037","volume":"78","author":"C Briand","year":"2019","unstructured":"Briand C, Fr\u00e9mond ML, Bessis D, et al. Efficacy of JAK1\/2 inhibition in the treatment of chilblain lupus due to TREX1 deficiency. Ann Rheum Dis. 2019;78(3):431\u20133.","journal-title":"Ann Rheum Dis"},{"issue":"1","key":"517_CR11","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1016\/j.jaci.2018.02.045","volume":"142","author":"K Meesilpavikkai","year":"2018","unstructured":"Meesilpavikkai K, Dik WA, Schrijver B, et al. Baricitinib treatment in a patient with a gain-of-function mutation in signal transducer and activator of transcription 1 (STAT1). J Allergy Clin Immunol. 2018;142(1):328-330.e2.","journal-title":"J Allergy Clin Immunol"},{"issue":"5","key":"517_CR12","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1002\/art.40805","volume":"71","author":"K Meesilpavikkai","year":"2019","unstructured":"Meesilpavikkai K, Dik WA, Schrijver B, et al. Efficacy of baricitinib in the treatment of chilblains associated with aicardi-gouti\u00e8res syndrome, a type I interferonopathy. Arthritis Rheumatol. 2019;71(5):829\u201331.","journal-title":"Arthritis Rheumatol"},{"issue":"5","key":"517_CR13","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1007\/s10875-019-00645-0","volume":"39","author":"S Volpi","year":"2019","unstructured":"Volpi S, Insalaco A, Caorsi R, et al. Efficacy and adverse events during janus kinase inhibitor treatment of SAVI syndrome. J Clin Immunol. 2019;39(5):476\u201385.","journal-title":"J Clin Immunol"},{"issue":"5","key":"517_CR14","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1007\/s00431-015-2668-4","volume":"175","author":"MP Cavalcante","year":"2016","unstructured":"Cavalcante MP, Brunelli JB, Miranda CC, et al. CANDLE syndrome: chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature-a rare case with a novel mutation. Eur J Pediatr. 2016;175(5):735\u201340.","journal-title":"Eur J Pediatr"},{"issue":"1","key":"517_CR15","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1038\/nrrheum.2015.167","volume":"12","author":"DM Schwartz","year":"2016","unstructured":"Schwartz DM, Bonelli M, Gadina M, O\u2019Shea JJ. Type I\/II cytokines, JAKs, and new strategies for treating autoimmune diseases. Nat Rev Rheumatol. 2016;12(1):25\u201336.","journal-title":"Nat Rev Rheumatol"},{"issue":"4","key":"517_CR16","doi-asserted-by":"publisher","first-page":"371","DOI":"10.1002\/jrsm.1123","volume":"5","author":"MT Pham","year":"2014","unstructured":"Pham MT, Raji\u0107 A, Greig JD, Sargeant JM, Papadopoulos A, Mcewen SA. A scoping review of scoping reviews: advancing the approach and enhancing the consistency. Res Synth Methods. 2014;5(4):371\u201385.","journal-title":"Res Synth Methods"},{"issue":"5","key":"517_CR17","doi-asserted-by":"publisher","first-page":"e028303","DOI":"10.1136\/bmjopen-2018-028303","volume":"9","author":"F G\u00f3mez-Garc\u00eda","year":"2019","unstructured":"G\u00f3mez-Garc\u00eda F, G\u00f3mez-Arias PJ, Hernandez J, et al. Drugs targeting the JAK\/STAT pathway for the treatment of immune-mediated inflammatory skin diseases: protocol for a scoping review. BMJ Open. 2019;9(5):e028303.","journal-title":"BMJ Open"},{"key":"517_CR18","unstructured":"Joanna Briggs Institute. The Joanna Briggs Institute reviewers\u2019 manual 2015: Methodology for JBI scoping reviews. 2015. http:\/\/joannabriggs.org\/assets\/docs\/sumari\/ReviewersManual_Mixed-Methods-Review-Methods-2014-ch1.pdf."},{"issue":"7","key":"517_CR19","doi-asserted-by":"publisher","first-page":"467","DOI":"10.7326\/M18-0850","volume":"169","author":"AC Tricco","year":"2018","unstructured":"Tricco AC, Lillie E, Zarin W, et al. PRISMA extension for scoping reviews (PRISMA-ScR): checklist and explanation. Ann Intern Med. 2018;169(7):467\u201373.","journal-title":"Ann Intern Med"},{"issue":"1","key":"517_CR20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s12969-019-0322-9","volume":"17","author":"M Boyadzhiev","year":"2019","unstructured":"Boyadzhiev M, Marinov L, Boyadzhiev V, Iotova V, Aksentijevich I, Hambleton S. Disease course and treatment effects of a JAK inhibitor in a patient with CANDLE syndrome. Pediatr Rheumatol. 2019;17(1):1\u20137.","journal-title":"Pediatr Rheumatol"},{"issue":"Suppl. 10","key":"517_CR21","first-page":"3208","volume":"69","author":"GAM Sanchez","year":"2017","unstructured":"Sanchez GAM, Reinhardt A, Ramsey S, et al. Response to JAK1\/2 inhibition with baricitinib in \u201cCANDLE\u201d, \u201cSAVI\u201d and \u201cCANDLE-like\u201d diseases. A new therapeutic approach for type I IFN-mediated autoinflammatory diseases. Arthritis Rheumatol. 2017;69(Suppl. 10):3208.","journal-title":"Arthritis Rheumatol."},{"issue":"7","key":"517_CR22","doi-asserted-by":"publisher","first-page":"3041","DOI":"10.1172\/JCI98814","volume":"128","author":"GA Sanchez-Montalegre","year":"2018","unstructured":"Sanchez-Montalegre GA, Reinhardt A, Ramsey S, et al. JAK1\/2 inhibition with baricitinib in the treatment of autoinflammatory interferonopathies. J Clin Invest. 2018;128(7):3041\u201352.","journal-title":"J Clin Invest"},{"issue":"3","key":"517_CR23","doi-asserted-by":"publisher","first-page":"342","DOI":"10.1001\/jamadermatol.2018.5077","volume":"155","author":"N Zimmermann","year":"2019","unstructured":"Zimmermann N, Wolf C, Schwenke R, et al. Assessment of clinical response to janus kinase inhibition in patients with familial chilblain lupus and TREX1 mutation. JAMA Dermatol. 2019;155(3):342\u20136.","journal-title":"JAMA Dermatol"},{"issue":"6","key":"517_CR24","doi-asserted-by":"publisher","first-page":"1752","DOI":"10.1016\/j.jaci.2016.07.015","volume":"138","author":"ML Fr\u00e9mond","year":"2016","unstructured":"Fr\u00e9mond ML, Rodero MP, Jeremiah N,et al. Efficacy of the Janus kinase 1\/2 inhibitor ruxolitinib in the treatment of vasculopathy associated with TMEM173-activating mutations in 3 children. J Allergy Clin Immunol. 2016;138(6):1752\u20135.","journal-title":"J Allergy Clin Immunol"},{"issue":"12","key":"517_CR25","doi-asserted-by":"publisher","first-page":"2241","DOI":"10.1093\/rheumatology\/kex316","volume":"56","author":"MN Manoussakis","year":"2017","unstructured":"Manoussakis MN, Mavragani CP, Nezos A, Zampeli E, Germenis A, Moutsopoulos HM. Type I interferonopathy in a young adult. Rheumatology (Oxford). 2017;56(12):2241\u20133.","journal-title":"Rheumatology (Oxford)"},{"issue":"Suppl 2","key":"517_CR26","first-page":"65","volume":"15","author":"S Volpi","year":"2017","unstructured":"Volpi S. Efficacy of the JAK inhibitor ruxolitinib in two patients with SAVI syndrome [abstract]. Pediatr Rheumatol Online J. 2017;15(Suppl 2):65.","journal-title":"Pediatr Rheumatol Online J"},{"issue":"Suppl 2","key":"517_CR27","first-page":"52","volume":"16","author":"AM Galimberti","year":"2018","unstructured":"Galimberti AM, Wiel LC, Gortani G, et al. Response to tofacitinib in a case with familiar CANDLE-like disease. Pediatr Rheumatol. 2018;16(Suppl 2):52.","journal-title":"Pediatr Rheumatol"},{"issue":"2","key":"517_CR28","doi-asserted-by":"publisher","first-page":"468","DOI":"10.1136\/annrheumdis-2016-209841","volume":"76","author":"N K\u00f6nig","year":"2017","unstructured":"K\u00f6nig N, Fiehn C, Wolf C, et al. Familial chilblain lupus due to a gain-of-function mutation in STING. Ann Rheum Dis. 2017;76(2):468\u201372.","journal-title":"Ann Rheum Dis"},{"issue":"Suppl 2","key":"517_CR29","first-page":"109","volume":"15","author":"S Rodionovskaya","year":"2017","unstructured":"Rodionovskaya S, Zaytseva S, Salugina S, Fedorov E, Tsymbal I. Successful use of tofacitinib in a 6-year-old patient with SAVI syndrome. Pediatr Rheumatol. 2017;15(Suppl 2):109\u201310.","journal-title":"Pediatr Rheumatol"},{"issue":"4","key":"517_CR30","doi-asserted-by":"publisher","first-page":"1396","DOI":"10.1016\/j.jaci.2016.10.030","volume":"139","author":"J Seo","year":"2017","unstructured":"Seo J, Kang JA, Suh DI, et al. Tofacitinib relieves symptoms of stimulator of interferon genes (STING)\u2013associated vasculopathy with onset in infancy caused by 2 de novo variants in TMEM173. J Allergy Clin Immunol. 2017;139(4):1396-1399.e12.","journal-title":"J Allergy Clin Immunol"},{"issue":"1","key":"517_CR31","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1007\/s10875-019-00690-9","volume":"40","author":"X Tang","year":"2020","unstructured":"Tang X, Xu H, Zhou C, et al. STING-Associated vasculopathy with onset in infancy in three children with new clinical aspect and unsatisfactory therapeutic responses to tofacitinib. J Clin Immunol. 2020;40(1):114\u201322.","journal-title":"J Clin Immunol"},{"issue":"4","key":"517_CR32","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1089\/jir.2017.0127","volume":"38","author":"H Kim","year":"2018","unstructured":"Kim H, De Jesus AA, Brooks SR, et al. Development of a validated interferon score using nanostring technology. J Interf Cytokine Res. 2018;38(4):171\u201385.","journal-title":"J Interf Cytokine Res"},{"issue":"4","key":"517_CR33","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1016\/j.rdc.2013.08.001","volume":"39","author":"GA Sanchez","year":"2013","unstructured":"Sanchez GA, de Jesus AA, Goldbach-Mansky R. Monogenic autoinflammatory diseases: disorders of amplified danger sensing and cytokine dysregulation. Rheum Dis Clin North Am. 2013;39(4):701\u201334.","journal-title":"Rheum Dis Clin North Am"},{"issue":"4","key":"517_CR34","doi-asserted-by":"publisher","first-page":"1669","DOI":"10.1172\/JCI129301","volume":"130","author":"AA de Jesus","year":"2020","unstructured":"de Jesus AA, Hou Y, Brooks S, et al. Distinct interferon signatures and cytokine patterns define additional systemic autoinflammatory diseases. J Clin Invest. 2020;130(4):1669\u201382.","journal-title":"J Clin Invest"},{"key":"517_CR35","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1016\/j.jclinepi.2017.08.004","volume":"92","author":"WJ Canestaro","year":"2017","unstructured":"Canestaro WJ, Hendrix N, Bansal A, Sullivan SD, Devine EB, Carlson JJ. Favorable and publicly funded studies are more likely to be published: a systematic review and meta-analysis. J Clin Epidemiol. 2017;92:58\u201368.","journal-title":"J Clin Epidemiol"}],"container-title":["Dermatology and Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13555-021-00517-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s13555-021-00517-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13555-021-00517-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,28]],"date-time":"2021-05-28T06:56:18Z","timestamp":1622184978000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s13555-021-00517-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,4,15]]},"references-count":35,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2021,6]]}},"alternative-id":["517"],"URL":"https:\/\/doi.org\/10.1007\/s13555-021-00517-9","relation":{},"ISSN":["2193-8210","2190-9172"],"issn-type":[{"value":"2193-8210","type":"print"},{"value":"2190-9172","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,4,15]]},"assertion":[{"value":"12 February 2021","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 April 2021","order":2,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}]}}