{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T01:58:31Z","timestamp":1785376711050,"version":"3.55.0"},"reference-count":43,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,2,10]],"date-time":"2026-02-10T00:00:00Z","timestamp":1770681600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,2,10]],"date-time":"2026-02-10T00:00:00Z","timestamp":1770681600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100017759","name":"Childhood Arthritis and Rheumatology Research Alliance","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100017759","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Chronic lung disease is a potentially life\u2010threatening complication of systemic juvenile idiopathic arthritis (SJIA\u2010LD). However, its natural history, etiology, and effective management are unclear. We aimed to describe the baseline characteristics and biomarker profiles of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry SJIA\u2010LD cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Baseline data from all CARRA Registry patients with SJIA and those with SJIA\u2010LD were included and analyzed. Lung disease\u2013specific data were obtained using a standardized case report form through REDCap Cloud. Plasma biomarker profiles were determined using a custom Luminex panel.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 37 patients with SJIA\u2010LD from 16 CARRA sites were identified and compared to all 928 patients with SJIA without known LD in the CARRA Registry. Patients with SJIA\u2010LD were significantly younger at disease onset and were more likely to be of Asian descent. A higher medication burden was also found. Patients with SJIA\u2010LD had higher levels of multiple lung injury biomarkers, cytokines, and chemokines compared to patients with both active and inactive SJIA without LD and healthy controls. Cluster analysis proposed three groups of SJIA\u2010LD patients with distinct biomarker patterns reflecting differences in proinflammatory cytokines, type II chemokines, and markers of macrophage activation syndrome (MAS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The CARRA SJIA\u2010LD Cohort exhibits distinct clinical features, higher medication burden, frequent MAS, and plasma biomarker patterns specific to SJIA\u2010LD compared to patients with SJIA without LD. A study is ongoing to assess more detailed clinical features, disease progression, patient\u2010reported outcomes, and associated immune biomarkers.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acr.70015","type":"journal-article","created":{"date-parts":[[2025,12,15]],"date-time":"2025-12-15T06:48:57Z","timestamp":1765781337000},"page":"1036-1048","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Baseline Clinical Features and Biomarker Profiles of the Childhood Arthritis and Rheumatology Research Alliance Systemic Juvenile Idiopathic Arthritis\u2010Associated Lung Disease Cohort"],"prefix":"10.1002","volume":"78","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4770-7383","authenticated-orcid":false,"given":"Esraa","family":"Eloseily","sequence":"first","affiliation":[{"name":"University of Texas Southwestern Medical Center  Dallas"},{"name":"Scottish Rite Hospital for Children  Dallas Texas"},{"name":"Faculty of Medicine Assiut University  Assiut Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary Ellen","family":"Riordan","sequence":"additional","affiliation":[{"name":"Joseph M. Sanzari Children's Hospital, Hackensack Meridian School of Medicine  Nutley New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ibrahim","family":"Mahmoud","sequence":"additional","affiliation":[{"name":"Azhar University Faculty of Medicine  Assiut Egypt"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Autumn","family":"Clark","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min\u2010Lee","family":"Chang","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Russell","sequence":"additional","affiliation":[{"name":"Duke Clinical Research Institute  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Natter","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sherry","family":"Thornton","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"},{"name":"University of Cincinnati  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3837-5337","authenticated-orcid":false,"given":"Scott","family":"Canna","sequence":"additional","affiliation":[{"name":"The Children's Hospital of Philadelphia  Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominic O.","family":"Co","sequence":"additional","affiliation":[{"name":"University of Wisconsin School of Medicine and Public Health  Madison"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marietta","family":"DeGuzman","sequence":"additional","affiliation":[{"name":"Baylor College of Medicine  Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7132-3390","authenticated-orcid":false,"given":"Yukiko","family":"Kimura","sequence":"additional","affiliation":[{"name":"Joseph M. Sanzari Children's Hospital, Hackensack Meridian School of Medicine  Nutley New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5923-7051","authenticated-orcid":false,"given":"Grant S.","family":"Schulert","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center  Cincinnati Ohio"},{"name":"University of Cincinnati  Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"CARRA Registry Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"CARRA FROST Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the CARRA Registry SJIA\u2010LD Cohort Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,10]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncprheum0084"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2011.68"},{"issue":"2","key":"e_1_2_7_4_1","first-page":"390","article-title":"International League of Associations for Rheumatology. International League of Associations for Rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001","volume":"31","author":"Petty RE","year":"2004","journal-title":"J Rheumatol"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2018.04.005"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev-med-061813-012806"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.41073"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.21889"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2019-216040"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2021-220578"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.24994"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.41888"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-017-0160-6"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.25234"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-022-00768-6"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1186\/ar3061"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39332"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780371209"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.42099"},{"issue":"9","key":"e_1_2_7_20_1","article-title":"Predictive biomarkers of tofacitinib response and disease activity in juvenile idiopathic arthritis subtypes: a longitudinal study [abstract]","volume":"74","author":"Ogbu E","year":"2022","journal-title":"Arthritis Rheumatol"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.26821"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-210324"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1520779112"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2352-4642(19)30027-6"},{"key":"e_1_2_7_25_1","article-title":"Key facts about Asian Americans, a diverse and growing population","volume":"1","author":"Krogstad JM","year":"2025","journal-title":"Pew Research Center. May"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-020-00444-7"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12098-019-02912-9"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab673"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2015.179"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39694"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2011.12.008"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2020-217470"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1172\/jci.insight.147593"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI129301"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.25129"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.25132"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2015.06.005"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.42137"},{"issue":"4","key":"e_1_2_7_39_1","first-page":"414","article-title":"Anti\u2010inflammatory drug development: broad or specific chemokine receptor antagonists?","volume":"13","author":"Russo RC","year":"2010","journal-title":"Curr Opin Drug Discov Devel"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1398-9995.2002.02166.x"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1159\/000500576"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cyto.2012.05.019"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2017-12-820852"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10875-024-01797-4"}],"container-title":["Arthritis Care &amp; Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr.70015","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr.70015","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr.70015","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T01:15:43Z","timestamp":1785374143000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr.70015"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,10]]},"references-count":43,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2026,8]]}},"alternative-id":["10.1002\/acr.70015"],"URL":"https:\/\/doi.org\/10.1002\/acr.70015","archive":["Portico"],"relation":{},"ISSN":["2151-464X","2151-4658"],"issn-type":[{"value":"2151-464X","type":"print"},{"value":"2151-4658","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,10]]},"assertion":[{"value":"2025-06-28","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-18","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}