Journal article
The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin‐1 Mediated Autoinflammatory Diseases: Cryopyrin‐Associated Periodic Syndromes, Tumour Necrosis Factor Receptor‐Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin‐1 Receptor Antagonist
Arthritis & rheumatology (Hoboken, N.J.), Vol.74, pp.1102-1121
05/27/2022
DOI: 10.1002/art.42139
PMCID: PMC9531906
PMID: 35623638
Abstract
BackgroundThe interleukin-1 (IL-1) mediated systemic autoinflammatory diseases, including the cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD) and deficiency of the IL-1 receptor antagonist (DIRA), belong to a group of rare immunodysregulatory diseases that primarily present in early childhood with variable multiorgan involvement. 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-1 have been life changing and have significantly improved patient outcomes.ObjectiveTo establish evidence-based recommendations for diagnosis, treatment and monitoring of patients with IL-1 mediated autoinflammatory diseases to standardise their management.MethodsA 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.ResultsThe task force devised five overarching principles, 14 statements related to diagnosis, 10 on therapy, and nine focused on long-term monitoring that were evidence and/or consensus-based for patients with IL-1 mediated diseases. An outline was developed for disease-specific monitoring of inflammation-induced organ damage progression and reported treatments of CAPS, TRAPS, MKD and DIRA.ConclusionThe 2021 EULAR/American College of Rheumatology points to consider represent state-of-the-art 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.
Details
- Title: Subtitle
- The 2021 EULAR/American College of Rheumatology Points to Consider for Diagnosis, Management and Monitoring of the Interleukin‐1 Mediated Autoinflammatory Diseases: Cryopyrin‐Associated Periodic Syndromes, Tumour Necrosis Factor Receptor‐Associated Periodic Syndrome, Mevalonate Kinase Deficiency, and Deficiency of the Interleukin‐1 Receptor Antagonist
- Creators
- Micol Romano - Western UniversityZ. Serap AriciDavid Piskin - Western UniversitySara Alehashemi - National Institute of Allergy and Infectious DiseasesDaniel Aletaha - Medical University of ViennaKaryl Barron - National Institute of Allergy and Infectious DiseasesSusanne Benseler - University of CalgaryRoberta A. Berard - Western UniversityLori Broderick - Rady Children's Hospital-San DiegoFatma Dedeoglu - Harvard UniversityMichelle Diebold - Western UniversityKaren Durrant - Kaiser Foundation HospitalPolly Ferguson - University of IowaDirk Foell - University of MünsterJonathan S. Hausmann - Beth Israel Deaconess Medical CenterOlcay Y. Jones - Walter Reed National Military Medical CenterDaniel Kastner - National Human Genome Research InstituteHelen J. Lachmann - University College LondonRonald M. Laxer - University of TorontoDorelia RiveraNicolino Ruperto - Istituto Giannina GasliniAnna Simon - Radboud University NijmegenMarinka Twilt - University of CalgaryJoost Frenkel - Wilhelmina Children's HospitalHal M. Hoffman - Rady Children's Hospital-San DiegoAdriana A. de Jesus - National Institute of Allergy and Infectious DiseasesJasmin B. Kuemmerle-DeschnerSeza Ozen - Hacettepe UniversityMarco Gattorno - Istituto Giannina GasliniRaphaela Goldbach-ManskyErkan Demirkaya - Western University
- Resource Type
- Journal article
- Publication Details
- Arthritis & rheumatology (Hoboken, N.J.), Vol.74, pp.1102-1121
- DOI
- 10.1002/art.42139
- PMID
- 35623638
- PMCID
- PMC9531906
- NLM abbreviation
- Arthritis Rheumatol
- ISSN
- 2326-5191
- eISSN
- 2326-5205
- Publisher
- Wiley
- Grant note
- DOI: 10.13039/501100008741, name: EULAR; DOI: 10.13039/100000002, name: NIH; DOI: 10.13039/100000060, name: NIAID; DOI: 10.13039/100000051, name: NHGRI; DOI: 10.13039/100000069, name: NIAMS
- Language
- English
- Date published
- 05/27/2022
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984531658002771
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