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Background : Interleukin- 1 ( IL-1 )-mediated systemic autoinflammatory diseases, including cold porphyrin-associated periodic syndrome ( CAPS ), tumor necrosis factor receptor-associated periodic syndrome ( TRAPS ), onychomycosis Valonate Kinase Deficiency ( MKD ) and IL-1 Receptor Antagonist Deficiency (DIRA) , belong to a group of rare immune dysregulation disorders that mainly present in early childhood with multiorgan involveme.
Background Interleukin- 1 ( IL-1 ) mediated systemic autoinflammatory diseases including cold porphyrin-associated periodic syndrome ( CAPS ), tumor necrosis factor receptor-associated periodic syndrome ( TRAPS ), Valerate kinase deficiency ( MKD ) and IL-1 receptor antagonist deficiency (DIRA) , belong to a group of rare immune dysregulation disorders that primarily present in early childhood with multiorgan involveme.
Objective To establish evidence-based recommendations for the diagnosis, treatment, and monitoring of patients with IL-1- mediated autoinflammatory diseases to standardize their manageme.
Methods A multinational, multidisciplinary working group of physician specialists was established, including rheumatologists, patients or caregivers, and relevant healthcare professiona.
RESULTS : The working group designed five overarching principles, 14 statements related to diagnosis, 10 statements about treatment, and 9 focused on long-term monitoring, all of which were evidence-based and / or consensus -based IL-1- mediated Surveillance of patients with disea.
Results The working group designed five overarching principles, 14 statements related to diagnosis, 10 statements about treatment, and 9 focused on long-term monitoring, all of which are evidence-based and / or consensus-based for IL-1- mediated disease patient monitori.
Conclusions: The 2021 EULAR/ American College of Rheumatology Essentials represents 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 standardize and improve care, quality of life and disease outcome.
Source: Romano M, Arici ZS, Piskin D , et a.
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 antagoni.
Annals of the Rheumatic Diseases2022;81:907-92 , et al Annals of the Rheumatic Diseasesleave a message here
