{"doi":"10.1002/art.42087","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: <scp>CANDLE</scp>/<scp>PRAAS</scp>, <scp>SAVI</scp>, and <scp>AGS</scp>","abstract":"OBJECTIVE: Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), and Aicardi-Goutières syndrome (AGS) are rare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targeted treatments, a Task Force was charged with the development of \"points to consider\" to improve diagnosis, treatment, and long-term monitoring of patients with these rare diseases. METHODS: 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, \"points to consider\" to guide patient management were developed. RESULTS: The Task Force devised consensus and evidence-based guidance of 4 overarching principles and 17 points to consider regarding the diagnosis, treatment, and long-term monitoring of patients with the autoinflammatory interferonopathies, CANDLE/PRAAS, SAVI, and AGS. CONCLUSION: These points to consider represent state-of-the-art 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.","journal":"Arthritis & Rheumatology","year":2022,"id":239939,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":47,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.961,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2022-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":866452,"name":"Lovro Lamot","orcid":"0000-0002-7939-115X","position":1,"is_corresponding":false},{"id":768148,"name":"Micol Romano","orcid":"0000-0001-7823-2563","position":2,"is_corresponding":false},{"id":768151,"name":"Erkan Demirkaya","orcid":"0000-0003-4525-1789","position":3,"is_corresponding":false},{"id":768149,"name":"David Piskin","orcid":"0000-0002-2315-514X","position":4,"is_corresponding":false},{"id":297408,"name":"Sofia Torreggiani","orcid":"0000-0003-0439-4046","position":5,"is_corresponding":false},{"id":274380,"name":"Laura Adang","orcid":"0000-0002-8454-1116","position":6,"is_corresponding":false},{"id":765948,"name":"Thaís Armangué","orcid":"0000-0001-6192-6171","position":7,"is_corresponding":false},{"id":866952,"name":"Kathe Barchus","orcid":null,"position":8,"is_corresponding":false},{"id":866953,"name":"Devon Cordova","orcid":null,"position":9,"is_corresponding":false},{"id":274413,"name":"Yanick J. 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