Browsing by Author "Adang, Laura A."

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    The 2021 European Alliance of Associations for Rheumatology/American College of Rheumatology Points to Consider for Diagnosis and Management of Autoinflammatory Type I Interferonopathies: CANDLE/PRAAS, SAVI, and AGS
    (2022) Gedik, Kader Cetin; Lamot, Lovro; Romano, Micol; Demirkaya, Erkan; Piskin, David; Torreggiani, Sofia; Adang, Laura A.; Armangue, Thais; Barchus, Kathe; Cordova, Devon R.; Crow, Yanick J.; Dale, Russell C.; Durrant, Karen L.; Eleftheriou, Despina; Fazzi, Elisa M.; Gattorno, Marco; Gavazzi, Francesco; Hanson, Eric P.; Lee-Kirsch, Min Ae; Montealegre Sanchez, Gina A.; Neven, Bénédicte; Orcesi, Simona; Ozen, Seza; Poli, Cecilia; Schumacher, Elliot; Tonduti, Davide; Uss, Katsiaryna; Aletaha, Daniel; Feldman, Brian M.; Vanderver, Adeline; Brogan, Paul A.; Goldbach-Mansky, Raphaela
    Objective.Autoinflammatory type I interferonopathies, chronic atypical neutrophilic dermatosis with lipodystrophyand elevated temperature/proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS), stimulator of inter-feron genes (STING)–associated vasculopathy with onset in infancy (SAVI), and Aicardi-Goutières syndrome (AGS) arerare and clinically complex immunodysregulatory diseases. With emerging knowledge of genetic causes and targetedtreatments, 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 literaturereview. Then, based on literature, Delphi questionnaires, and consensus methodology,“points to consider”to guidepatient management were developed.Results.The Task Force devised consensus and evidence-based guidance of 4 overarching principles and17 points to consider regarding the diagnosis, treatment, and long-term monitoring of patients with the autoinflamma-tory interferonopathies, CANDLE/PRAAS, SAVI, and AGS.Conclusion.These points to consider represent state-of-the-art knowledge to guide diagnostic evaluation, treat-ment, and management of patients with CANDLE/PRAAS, SAVI, and AGS and aim to standardize and improve care,quality of life, and disease outcomes.

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