{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T21:13:35Z","timestamp":1789593215428,"version":"build-2803163510"},"reference-count":56,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T00:00:00Z","timestamp":1772064000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T00:00:00Z","timestamp":1772064000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["Z01\u2010AR\u2010041198"],"award-info":[{"award-number":["Z01\u2010AR\u2010041198"]}],"id":[{"id":"10.13039\/100000069","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":[[2026,8]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To evaluate the relationship across type I interferon (IFN\u2010I)\u2013stimulated gene (ISG) expression, Still disease, and the development of lung disease (LD) and drug\u2010associated immune reactions (DAIR) to interleukin\u20101 (IL\u20101) and\/or IL\u20106 inhibitors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Whole blood ISG expression was quantified by NanoString array. ISG\u201028 scores were calculated in consecutive patients with Still or Still\u2010like disease. Exome sequencing with family\u2010based variant prioritization identified candidate genes harboring rare candidate causative variants. Lists of candidate genes were subjected to functional enrichment analysis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 57 patients (32 children, 25 adults), 16 had elevated ISG\u201028 scores. This group exhibited higher prevalence of LD (0.44 vs 0.1,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.007) and DAIR (0.63 vs 0.17,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.003) and lower IL\u20106 inhibitor use (0 vs 0.25,\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.048) compared to others. No significant differences were found in the rates of macrophage activation syndrome, active disease, elevated IL\u201018, or current IL\u20101 inhibition. The combination of HLA\u2010DRB1*15 with high ISG\u201028 scores is associated with LD and DAIR with high specificity, whereas absence of both biomarkers had high negative predictive value. Candidate genes from high ISG\u201028 individuals were enriched in IFN\u2010related pathways, including autophagy, IFN\u2010I production, toll\u2010like receptor signaling, macrophage activation, cytoskeletal organization, and responses to stress.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>High IFN\u2010I expression correlates with LD and DAIR in Still disease, linked to rare genetic variation in immune pathways. Combining high ISG\u201028 with HLA\u2010DRB1*15 significantly improves post hoc stratification of patients for these complications. If prospectively validated, these findings may guide molecular risk assessment and targeted therapies, including IFN\u2010I directed treatments in Still disease with IFN\u2010I signature.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.70079","type":"journal-article","created":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T10:36:09Z","timestamp":1770806169000},"page":"1776-1788","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Type I Interferon Signature is Associated With Lung Disease, Drug\u2010Associated Immune Reactions, and Genetic Variation in Interferon\u2010Linked Pathways in Still Disease"],"prefix":"10.1002","volume":"78","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1195-1595","authenticated-orcid":false,"given":"Mariana Correia","family":"Marques","sequence":"first","affiliation":[{"name":"Translational Genetics and Genomics Section, Intramural Research Program, National 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