{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T20:56:31Z","timestamp":1788555391299,"version":"build-2803163510"},"reference-count":14,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,4,22]],"date-time":"2025-04-22T00:00:00Z","timestamp":1745280000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","award":["75N91019D000024"],"award-info":[{"award-number":["75N91019D000024"]}],"id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>The objective was to report the safety and efficacy of an anti\u2010IFNAR1 antibody (anifrolumab) in a patient with STING\u2010associated vasculopathy with onset in infancy (SAVI) who presented with vasculitic ulcers and systemic inflammation refractory to JAK inhibition (JAKi) and to the interferon\u2010\u03b2\u2013neutralizing monoclonal antibody dazukibart.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      A patient with SAVI and a de novo\n                      <jats:italic>STING1<\/jats:italic>\n                      p.(Asn154Ser) mutation, a known pathogenic variant, and uncontrolled disease received 21 doses of dazukibart under a compassionate use investigational new drug protocol, which was followed by treatment with the anti\u2010IFNAR1 antibody anifrolumab. Clinical and laboratory parameters, including wound healing, whole\u2010blood type I interferon (IFN I) signature, and safety markers were closely monitored throughout both treatment periods.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Despite initial reductions in C\u2010reactive protein levels and IFN I scores following dazukibart administration, the patient experienced rebound inflammation and recurrent vasculitic lesions. Dazukibart dose adjustments failed to sustainably control IFN I signaling. Subsequent combination therapy of baricitinib and tocilizumab proved partially effective. Treatment with anifrolumab, an IFNAR1 blocker, in conjunction with tocilizumab led to sustained suppression of IFN I scores, allowed discontinuation of JAKi, and resulted in significant improvement in vasculitic wounds.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case underscores the challenges in treating patients with SAVI and highlights the utility of IFN I scores as a theragnostic biomarker. Although high\u2010dose JAKi and dazukibart failed to achieve sustained control of IFN I signaling, treatment with anifrolumab durably suppressed IFN scores and demonstrated promising efficacy, which allows for the investigation of the role of IFN I signaling in the disease pathogenesis of SAVI and other interferonopathies in future clinical trials.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.43145","type":"journal-article","created":{"date-parts":[[2025,3,11]],"date-time":"2025-03-11T09:15:03Z","timestamp":1741684503000},"page":"1087-1091","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Sustained Interferon Signature Suppression With Anifrolumab in a Patient With STING\u2010Associated Vasculopathy with Onset in Infancy Refractory to\n                    <scp>JAK<\/scp>\n                    Inhibitor and Dazukibart Therapy"],"prefix":"10.1002","volume":"77","author":[{"given":"Sara","family":"Alehashemi","sequence":"first","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bjoern","family":"Buehring","sequence":"additional","affiliation":[{"name":"Bergisches Rheuma\u2010Zentrum  Wuppertal Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana A.","family":"de Jesus","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sachin","family":"Gaurav","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andre","family":"Rastegar","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexi","family":"Baumgardner","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-3277-026X","authenticated-orcid":false,"given":"Kip","family":"Friend","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oluwatobi T.","family":"Arisa","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Program National Cancer Institute, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William D.","family":"Figg","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Program National Cancer Institute, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle","family":"Fink","sequence":"additional","affiliation":[{"name":"Frederick National Laboratory for Cancer Research  Frederick Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Douglas B.","family":"Kuhns","sequence":"additional","affiliation":[{"name":"Frederick National Laboratory for Cancer Research  Frederick Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben","family":"Colton","sequence":"additional","affiliation":[{"name":"Pharmacy Department National Institutes of Health Clinical Center, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cody J.","family":"Peer","sequence":"additional","affiliation":[{"name":"Clinical Pharmacology Program National Cancer Institute, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raphaela","family":"Goldbach\u2010Mansky","sequence":"additional","affiliation":[{"name":"Translational Autoinflammatory Diseases Section National Institute of Allergy and Infectious Diseases, NIH  Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,4,22]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1312625"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI98814"},{"issue":"10","key":"e_1_2_7_4_1","article-title":"Monitoring of BK reactivation and long\u2010term safety on JAK1\/2 inhibition with baricitinib [abstract]","volume":"72","author":"Cetin Gedik K","year":"2020","journal-title":"Arthritis Rheumatol"},{"key":"e_1_2_7_5_1","unstructured":"U.S. Food and Drug Administration. \u201cFDA requires warnings about increased risk of serious heart\u2010related events cancer blood clots and death for JAK inhibitors that treat certain chronic inflammatory conditions.\u201d U.S. Food and Drug Administration Drug Safety Communication 1 Sept. 2021.https:\/\/www.fda.gov\/drugs\/drug\u2010safety\u2010and\u2010availability\/fda\u2010requires\u2010warnings\u2010about\u2010increased\u2010risk\u2010serious\u2010heart\u2010related\u2010events\u2010cancer\u2010blood\u2010clots\u2010and\u2010death."},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/cpdd.887"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.139949"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.15006"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1912196"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/cpt.936"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bcp.2005.12.041"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aat3356"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jlr.2024.100568"},{"issue":"9","key":"e_1_2_7_14_1","article-title":"Anifrolumab normalizes 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