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EULAR points to consider can guide the diagnostic evaluation, treatment and monitoring of SAIDs
SAIDs are a group of multisystem diseases caused by malfunction of the innate immune system. A number of these SAIDs are mediated by a cytokine called interleukin-1 (IL-1), including cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), and IL-1 receptor antagonist deficiency (DIRA). These rare diseases usually begin in early childhood with variable multi-organ involvement. The new EULAR Points-to-Consider were developed by a multidisciplinary working group from 7 European countries. Participants had expertise in pediatric and adult rheumatology. The group also included healthcare professionals and patient representatives. The work was carried out in accordance with EULAR standardized operating procedures...
EULAR points to consider can guide the diagnostic evaluation, treatment and monitoring of SAIDs
SAIDs are a group of multisystem diseases caused by malfunction of the innate immune system. A number of these SAIDs are mediated by a cytokine called interleukin-1 (IL-1), including cryopyrin-associated periodic syndromes (CAPS), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), and IL-1 receptor antagonist deficiency (DIRA). These rare diseases usually begin in early childhood with variable multi-organ involvement.
The new EULAR Points-to-Consider were developed by a multidisciplinary working group from 7 European countries. Participants had expertise in pediatric and adult rheumatology. The group also included healthcare professionals and patient representatives. The work was carried out in accordance with EULAR standardized operating procedures. The aim was to create evidence-based recommendations for the diagnosis, treatment and monitoring of people with IL-1-mediated autoinflammatory diseases.
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The paper, developed by EULAR and published in the 2022 issue of the Annals of the Rheumatic Diseases, sets out five overarching principles and three points to consider. The principles state that a multidisciplinary team is required to evaluate and treat people with IL-1-mediated SAIDs and that patients presenting with flares of unexplained systemic inflammation should receive an immediate diagnostic workup including genetic testing and screening for complications. The goal of treatment is to control clinical signs and symptoms and normalize inflammation using a treat-to-target approach and requires long-term monitoring.
The three points to be considered include 14 statements on diagnosis, 10 on therapy and 9 on long-term monitoring. For diagnosis, the points include the genetic and clinical clarification of certain diseases. The therapy statements provide recommendations for the use of IL-1 blocker therapy, each supported by a level of recommendation and evidence. The 9 points to consider in monitoring concern disease activity and burden, as well as the growth and development of children with SAIDs and the risk of complications and infections. These also include recommendations for vaccinations.
EULAR believes that these points to consider can guide the diagnostic evaluation, treatment and monitoring of patients and help standardize and improve care, quality of life and disease outcomes.
Source:
European Alliance of Rheumatology Associations, EULAR
Reference:
Romano, M., et al. (2022) The 2021 EULAR/American College of Rheumatology indicates that the following should be considered in the diagnosis, treatment and monitoring of the interleukin-1-mediated autoinflammatory diseases: cryopyrin-associated periodic syndrome, tumor necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency and interleukin-1 receptor antagonist deficiency. Annals of rheumatic diseases. doi.org/10.1136/annrheumdis-2021-221801.
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