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When untreated, patients with severe clinical phenotypes have a poor prognosis, and diagnosis and management of these patients can be challenging. However, approved treatments targeting the proinflammatory cytokine IL\u20101 have been life changing and have significantly improved patient outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>To establish evidence\u2010based recommendations for diagnosis, treatment and monitoring of patients with IL\u20101 mediated autoinflammatory diseases to standardise their management.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A multinational, multidisciplinary task force consisting of physician experts, including rheumatologists, patients or caregivers and allied healthcare professionals, was established. Evidence synthesis, including systematic literature review and expert consensus (Delphi) via surveys, was conducted. Consensus methodology was used to formulate and vote on statements to guide optimal patient care.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The task force devised five overarching principles, 14 statements related to diagnosis, 10 on therapy, and nine focused on long\u2010term monitoring that were evidence and\/or consensus\u2010based for patients with IL\u20101 mediated diseases. An outline was developed for disease\u2010specific monitoring of inflammation\u2010induced organ damage progression and reported treatments of CAPS, TRAPS, MKD and DIRA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The 2021 EULAR\/American College of Rheumatology points to consider represent state\u2010of\u2010the\u2010art knowledge based on published data and expert opinion to guide diagnostic evaluation, treatment and monitoring of patients with CAPS, TRAPS, MKD and DIRA, and to standardise and improve care, quality of life and disease outcomes.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.42139","type":"journal-article","created":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T07:18:01Z","timestamp":1653635881000},"page":"1102-1121","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":51,"title":["The 2021 EULAR\/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin\u20101 Mediated Autoinflammatory Diseases: Cryopyrin\u2010Associated Periodic Syndromes, Tumour Necrosis Factor Receptor\u2010Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin\u20101 Receptor Antagonist"],"prefix":"10.1002","volume":"74","author":[{"given":"Micol","family":"Romano","sequence":"first","affiliation":[{"name":"University of Western Ontario  London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Z. 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