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This study was undertaken to retrospectively evaluate incidence and risk factors for eosinophilia and describe lung disease outcomes in IL\u20101\/IL\u20106 inhibitor\u2013exposed patients with systemic JIA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Among JIA patients at our institution exposed to interleukin\u20101 (IL\u20101)\/IL\u20106 inhibitors (1995\u20132022), we compared incidence rate of eosinophilia in systemic JIA compared to other JIA, stratified by medication class (IL\u20101\/IL\u20106 inhibitors, other cytokine inhibitors, methotrexate). We used Cox models to identify predictors of eosinophilia during IL\u20101\/IL\u20106 inhibitor use and summarized treatment changes and outcomes after eosinophilia, including lung disease. HLA typing was performed on a clinical or research basis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>There were 264 new medication exposures in 75 patients with systemic JIA and 41 patients with other JIA. A total of 49% of patients with systemic JIA with HLA typing (n\u00a0=\u00a045) were positive for HLA\u2013DRB1*15 alleles. Eosinophilia was common during IL\u20101\/IL\u20106 inhibitor use and did not differ by systemic JIA compared to other JIA (0.08 and 0.07 per person\u2010year, respectively; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.30). Among systemic JIA patients, pretreatment macrophage activation syndrome (MAS) was associated with a higher rate of subsequent eosinophilia on biologic therapy (unadjusted hazard ratio 3.2 [95% confidence interval 1.2\u20138.3]). A total of 4 of 5 patients who switched therapy within 10\u2009weeks of eosinophilia experienced disease flare compared to none of the patients who continued the original therapy. A total of 8 of 25 patients with pulmonary evaluations had lung disease, and all had severe manifestations of systemic JIA (MAS, intensive care unit stay). One death was attributed to systemic JIA\u2013lung disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Eosinophilia is common in JIA patients using IL\u20101\/IL\u20106 inhibitors. Severe disease may be associated with eosinophilia and lung disease in systemic JIA.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.25129","type":"journal-article","created":{"date-parts":[[2023,4,11]],"date-time":"2023-04-11T04:35:30Z","timestamp":1681187730000},"page":"2063-2072","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Incidence and Risk Factors for Eosinophilia and Lung Disease in <scp>Biologic\u2010Exposed<\/scp> Children With Systemic Juvenile Idiopathic Arthritis"],"prefix":"10.1002","volume":"75","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8978-3184","authenticated-orcid":false,"given":"Holly","family":"Wobma","sequence":"first","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sage R.","family":"Arvila","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5761-7365","authenticated-orcid":false,"given":"Maria L.","family":"Taylor","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ki Pui","family":"Lam","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina","family":"Ohashi","sequence":"additional","affiliation":[{"name":"HLA\u2010Lab, American Red Cross  Dedham Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Gebhart","sequence":"additional","affiliation":[{"name":"HLA\u2010Lab, American Red Cross  Dedham Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helene","family":"Powers","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siobhan","family":"Case","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital and Brigham &amp; 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