{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T07:30:57Z","timestamp":1790753457048,"version":"4.1.0"},"reference-count":96,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,1,2]],"date-time":"2020-01-02T00:00:00Z","timestamp":1577923200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["DFG FO 354\/11\u20101"],"award-info":[{"award-number":["DFG FO 354\/11\u20101"]}],"id":[{"id":"10.13039\/501100001659","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":[[2020,2]]},"abstract":"<jats:p>Systemic juvenile idiopathic arthritis (<jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content>) is a form of arthritis in childhood that is initially dominated by innate immunity\u2013driven systemic inflammation and is thus considered a polygenic autoinflammatory disease. However, systemic <jats:styled-content style=\"fixed-case\">JIA<\/jats:styled-content> can progress toward an adaptive immunity\u2013driven afebrile arthritis. Based on this observation of biphasic disease progression, a \u201cwindow of opportunity\u201d for optimal, individualized and target\u2010directed treatment has been proposed. This hypothesis requires testing, and in this review we summarize current evidence regarding molecular factors that may contribute to the progression from an initially predominantly autoinflammatory disease phenotype to autoimmune arthritis. We consider the involvement of innately adaptive \u03b3\u03b4 T cells and natural killer T cells that express \u03b3\u03b4 or \u03b1\u03b2 T cell receptors but cannot be classified as either purely innate or adaptive cells, versus classic B and T lymphocytes in this continuum. Finally, we discuss our understanding of how and why some primarily autoinflammatory conditions can progress toward autoimmune\u2010mediated disorders over the disease course while others do not and how this knowledge may be used to offer individualized treatment.<\/jats:p>","DOI":"10.1002\/art.41107","type":"journal-article","created":{"date-parts":[[2019,9,16]],"date-time":"2019-09-16T05:42:42Z","timestamp":1568612562000},"page":"210-219","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Innately Adaptive or Truly Autoimmune: Is There Something Unique About Systemic Juvenile Idiopathic Arthritis?"],"prefix":"10.1002","volume":"72","author":[{"given":"Christoph","family":"Kessel","sequence":"first","affiliation":[{"name":"University of Munster  Munster Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1295-6179","authenticated-orcid":false,"given":"Christian 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