Treatment with the interleukin-1 inhibitor rilonacept (Arcalyst) resulted in rapid clinical improvements in systemic juvenile idiopathic arthritis patients, supporting the emerging concept of cytokine
The striking efficacy of these cytokine inhibitors has prompted some authorities to suggest that the classification of systemic JIA should be reconsidered.
“Systemic JIA is likely best classified as an autoinflammatory disease, based upon the robust clinical response to IL-1 inhibition and the absence of known autoantibodies, autoreactive T-cells, or association with HLA genotypes,” explainedTimothy Beukelman, MD, of the University of Alabama at Birmingham, who was a co-author of the updated ACR guidelines.
The autoinflammatory syndromes include conditions such as CAPS, familial Mediterranean fever, and TNF-receptor-associated periodic syndrome, which are associated with inflammation and recurring fevers among children.
These syndromes primarily involve the innate, rather than the adaptive, immune system.
Been saying this for years