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With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of \u201cpoints to consider\u201d to improve diagnosis, treatment, and long\u2010term monitoring of patients with these rare diseases.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n Members of a Task Force consisting of rheumatologists, neurologists, an immunologist, geneticists, patient advocates, and an allied health care professional formulated research questions for a systematic literature review. Then, based on literature, Delphi questionnaires, and consensus methodology, \u201cpoints to consider\u201d to guide patient management were developed.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n The Task Force devised consensus and evidence\u2010based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment, and long\u2010term monitoring of patients with the autoinflammatory interferonopathies, CANDLE\/PRAAS, SAVI, and AGS.<\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n These points to consider represent state\u2010of\u2010the\u2010art knowledge to guide diagnostic evaluation, treatment, and management of patients with CANDLE\/PRAAS, SAVI, and AGS and aim to standardize and improve care, quality of life, and disease outcomes.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/art.42087","type":"journal-article","created":{"date-parts":[[2022,3,22]],"date-time":"2022-03-22T00:01:49Z","timestamp":1647907309000},"page":"735-751","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":50,"title":["The 2021 European Alliance of Associations for Rheumatology\/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies:\n CANDLE<\/scp>\n \/\n PRAAS<\/scp>\n ,\n SAVI<\/scp>\n , and\n AGS<\/scp>"],"prefix":"10.1002","volume":"74","author":[{"given":"Kader","family":"Cetin Gedik","sequence":"first","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases NIH Bethesda Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lovro","family":"Lamot","sequence":"additional","affiliation":[{"name":"University of Zagreb School of Medicine Zagreb Croatia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micol","family":"Romano","sequence":"additional","affiliation":[{"name":"University of Western Ontario London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erkan","family":"Demirkaya","sequence":"additional","affiliation":[{"name":"University of Western Ontario London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Piskin","sequence":"additional","affiliation":[{"name":"University of Western Ontario, London Health Sciences Center, and Lawson Health Research Institute London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sofia","family":"Torreggiani","sequence":"additional","affiliation":[{"name":"National Institute of Allergy and Infectious Diseases NIH, Bethesda, Maryland, and UOC Pediatria a Media Intensit\u00e0 di Cura, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura A.","family":"Adang","sequence":"additional","affiliation":[{"name":"Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thais","family":"Armangue","sequence":"additional","affiliation":[{"name":"Sant Joan de Deu Children's Hospital and IDIBAPS\u2013Hospital Clinic; 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