POS1372 THE 2021 EULAR AND ACR POINTS TO CONSIDER FOR DIAGNOSIS, MANAGEMENT AND MONITORING OF THE IL 1 MEDIATED AUTOINFLAMMATORY DISEASES: CAPS, TRAPS, MKD, AND DIRA

Background The Interleukin-1 (IL-1) mediated systemic autoinflammatory diseases (SAIDs), including the cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor-associated syndrome (TRAPS), mevalonate kinase deficiency (MKD), and of IL-1 receptor antagonist (DIRA) belong to a group rare immunodysregulatory that primarily present in early childhood with variable multiorgan involvement. When untreated, patients severe clinical phenotypes have poor prognosis, diagnosis management these can be challenging. However, recently-approved treatments targeting pro-inflammatory cytokine been life-changing significantly improved patient outcomes. Objectives We aimed establish evidence-based recommendations on diagnosis, treatment, monitoring standardize patients. Methods A multinational, multidisciplinary task force consisting physician experts rheumatologists, or caregivers, allied health care professionals was established. Evidence synthesis systematic literature review expert consensus (Delphi) via surveys were conducted. Consensus methodology utilized formulate vote statements guide optimal care. Results devised five overarching principles, 14 related 10 therapy, 9 focused long-term evidence and/or consensus-based for diseases. An outline developed disease-specific inflammation-induced organ damage progression reported therapies CAPS, TRAPS, MKD, DIRA. Conclusion 2021 EULAR/ACR points consider provide state-of-the-art knowledge based published data opinion diagnostic evaluation, MKD DIRA, inform various stakeholders about optimized improve disease References [1]Masters SL, Simon A, Aksentijevich I, et al. Horror autoinflammaticus: molecular pathophysiology (*). Annu Rev Immunol 2009;27:621-68. Disclosure Interests micol romano: None declared, Zehra Serap Arici: David Piskin: Sara Alehashemi: Daniel Aletaha Speakers bureau: Lilly, Merck, Pfizer, Roche, Sandoz, Consultant of: Abbvie, Amgen, Novartis, Grant/research support from: SoBi, Sanofi, Karyl Barron: Susa Benseler: Roberta Berard Sandoz Lori Broderick Novartis Regeneron, Fatma Dedeoglu Michelle Diebold: Karen Durrant: Polly Ferguson NIH, CARRA, Inc, Dirk Foell Peer Voice Boehringer Ingelheim, Chugai-Roche, Jonathan Hausmann Biogen Olcay Jones: Kastner: Helen J. Lachmann: Ronald Laxer Dorelia Rivera: Nicolino Ruperto Eli-Lilly, GSK, Pfizer SoBi UCB, Ablynx, Astrazeneca-Medimmune, Aurinia, Bayer, Bristol Myers Squib, Cambridge Healthcare research, Celgene, Domain therapeutic, EMD Serono, Idorsia, Janssen, Anna Simon: Marinka Twilt: Joost Frenkel: Hal Hoffman Aclaris, Bristol-Meyer-Squib, Jecure, Takeda Zomagen, Adriana de Jesus: Jasmin Kuemmerle-Deschner Seza Özen Marco Gattorno Raphaela goldbach-mansky: Erkan Demirkaya Sobi