Cetin Gedik, KaderLamot, LovroRomano, MicolDemirkaya, ErkanPiskin, DavidTorreggiani, SofiaAdang, Laura A.Armangue, ThaisBarchus, KatheCordova, Devon R.Crow, Yanick J.Dale, Russell C.Durrant, Karen L.Eleftheriou, DespinaFazzi, Elisa M.Gattorno, MarcoGavazzi, FrancescoHanson, Eric P.Lee-Kirsch, Min AeMontealegre Sanchez, Gina A.Neven, BénédicteOrcesi, SimonaOzen, SezaPoli, M. CeciliaSchumacher, ElliotTonduti, DavideUss, KatsiarynaAletaha, DanielFeldman, Brian M.Vanderver, AdelineBrogan, Paul A.Goldbach-Mansky, Raphaela2024-01-082024-01-082022Cetin Gedik K, Lamot L, Romano M, et al. 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. Ann Rheum Dis. 2022;81(5):601-613. doi:10.1136/annrheumdis-2021-221814https://hdl.handle.net/1805/37699Objective: 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 healthcare 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 standardise and improve care, quality of life and disease outcomes.en-USPublisher PolicyType I interferonopathiesAutoimmune diseases of the nervous systemErythema nodosumFingersNervous system malformationsRheumatologySkin diseasesArticle