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

Abstract

BackgroundThe Interleukin-1 (IL-1) mediated systemic autoinflammatory diseases (SAIDs), including the cryopyrin-associated periodic syndromes (CAPS), tumor 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, recently-approved treatments targeting the pro-inflammatory cytokine IL-1 have been life-changing and have significantly improved patient outcomes.ObjectivesWe aimed to establish evidence-based recommendations on diagnosis, treatment, and monitoring to standardize the management of these patients.MethodsA multinational, multidisciplinary task force consisting of physician experts including rheumatologists, patients or caregivers, and allied health care professionals was established. Evidence synthesis including systematic literature review and expert consensus (Delphi) via surveys were conducted. Consensus methodology was utilized 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 9 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 therapies of CAPS, TRAPS, MKD, and DIRA.ConclusionThe 2021 EULAR/ACR points to consider provide 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 inform the various stakeholders about optimized patient care to improve disease outcomes.