{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T14:15:34Z","timestamp":1775830534073,"version":"3.50.1"},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2020,1,28]],"date-time":"2020-01-28T00:00:00Z","timestamp":1580169600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Bundesministerium f\u00fcr Bildung und Forschung","doi-asserted-by":"publisher","award":["01 GM 1512D"],"award-info":[{"award-number":["01 GM 1512D"]}],"id":[{"id":"10.13039\/501100002347","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":[[2020,3]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To analyze the reported association of <jats:italic>IL1RN<\/jats:italic> polymorphisms with response to interleukin\u20101 (IL\u20101) blockade in a German cohort of patients with systemic juvenile idiopathic arthritis (JIA), and to assess the impact of other factors on treatment response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sixty\u2010one patients with systemic JIA who had received IL\u20101 blockade were identified within the German Autoinflammatory Disease registry DNA biobank. Response to IL\u20101 blockade was assessed according to 1) the clinical response (initially at least a transient response or good response compared to a poor response), 2) switch (or no switch) to anti\u2013IL\u20106 receptor therapy following IL\u20101 blockade, 3) achievement of clinically inactive disease within 6 months of IL\u20101 blockade, 4) improvement in disease activity measured using the modified Juvenile Arthritis Disease Activity Score, and 5) achievement of a glucocorticoid\u2010free state. In addition, basic demographic data, key features of the disease course, laboratory data, and <jats:italic>IL1RN<\/jats:italic> single\u2010nucleotide polymorphisms (SNPs) were assessed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Six of 7 <jats:italic>IL1RN<\/jats:italic> SNPs reported to be associated with response to anakinra therapy were analyzed. These 6 <jats:italic>IL1RN<\/jats:italic> SNPs were inherited as haplotypes. An association of <jats:italic>IL1RN<\/jats:italic> haplotypes and SNPs with response to IL\u20101 blockade could not be confirmed in this cohort of patients with systemic JIA. Patients who received tocilizumab following IL\u20101 blockade had a longer duration from disease onset to diagnosis than those who did not receive tocilizumab (median 0.27 years versus 0.08 years).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The results of this study could not confirm an impact of <jats:italic>IL1RN<\/jats:italic> SNPs on response to IL\u20101 blockade therapy with either anakinra or canakinumab in a cohort of patients with systemic JIA. However, a longer time frame from disease onset to diagnosis was associated with poorer long\u2010term treatment response, thereby supporting the \u201cwindow of opportunity\u201d hypothesis that suggests improved long\u2010term treatment response with shorter time from disease onset to diagnosis (and treatment).<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.41130","type":"journal-article","created":{"date-parts":[[2019,10,10]],"date-time":"2019-10-10T03:40:07Z","timestamp":1570678807000},"page":"499-505","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Impact of <i>IL1RN<\/i> Variants on Response to Interleukin\u20101 Blocking Therapy in Systemic Juvenile Idiopathic Arthritis"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9247-4729","authenticated-orcid":false,"given":"Claas","family":"Hinze","sequence":"first","affiliation":[{"name":"University Hospital Munster  Munster 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