Interpretation on 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

Interleukin-1 mediated autoinflammatory diseases, a large class of autoinflammatory diseases characterized by increased release of interleukin-1 or activation of the interleukin-1 pathway, mainly include familial Mediterranean fever, cryopyrin-associated periodic syndrome, tumor necrosis factor receptor associated periodic syndrome, mevalonate kinase deficiency syndrome and deficiency of the interleukin-1 receptor antagonist. These diseases present in early childhood and affect multiple systems, which, if untreated, may result in progressive organ damage, morbidity and mortality. In 2016, European Alliance of Associations for Rheumatology (EULAR) published recommendations for familial Mediterranean fever. For the latter four types of diseases, EULAR and the American College of Rheumatology developed several points in terms of diagnosis, management and monitoring in 2022. This article aims to introduce the application of these points.