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
Abstract
The 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.
Document Details
- Document Type
- Pub Defense Publication
- Publication Date
- May 27, 2022
- Source ID
- 10.1002/art.42139
Entities
People
- Adriana A. De Jesus
- Anna J Simon
- Daniel Aletaha
- Daniel Kastner
- David Piskin
- Dirk Foell
- Dorelia Rivera
- Erkan Demirkaya
- Fatma Dedeoglu
- Hal M. Hoffman
- Helen J. Lachmann
- Jasmin Kümmerle-Deschner
- Jonathan Hausmann
- Joost Frenkel
- Karen Durrant
- Karyl Barron
- Lori Broderick
- Marco Gattorno
- Marinka Twilt
- Michelle Diebold
- Micol Romano
- Nicolino Ruperto
- Olcay Y Jones
- Polly Ferguson
- Raphaela T. Goldbach-Mansky
- Roberta A Berard
- Ronald M. Laxer
- Sara Alehashemi
- Seza Özen
- Susanne Benseler
- Z. Serap Arici
Organizations
- Boston Children's Hospital
- European Alliance of Associations for Rheumatology
- Hacettepe University
- Harvard Medical School
- Medical University of Vienna
- National Human Genome Research Institute
- National Institute of Allergy and Infectious Diseases
- National Institute of Arthritis and Musculoskeletal and Skin Diseases
- National Institutes of Health
- Radboud University Nijmegen
- Tübingen University Hospital
- University College London
- University of Calgary
- University of California
- University of Iowa
- University of Münster
- University of Toronto
- Walter Reed National Military Medical Center
- Western University