Review of Biologics in Children With Rheumatic Diseases

Shabina Habibi; Athimalaipet V Ramanan


Int J Clin Rheumatol. 2012;7(1):81-93. 

In This Article

IL-1 Blockers

SOJIA is characterized by spiking fever, often associated with rash, pericarditis, arthritis and hepatosplenomegaly. IL-1β is a proinflammatory cytokine, synthesized by monocytes/macrophages and dendritic cells. It induces systemic features such as fever, and also the production of other proinflammatory mediators such as prostaglandins, IL-6 and TNF-α. Its effects are downregulated by a receptor antagonist, IL-1RA, which binds to the IL-1 receptor, but does not cause downstream stimulation. This cytokine is thought to play a major role in the pathogenesis of SOJIA, along with IL-6. Mononuclear cells from children with SOJIA spontaneously produce large amounts of IL-1, and serum samples from these children were found to induce production of IL-1 from healthy peripheral blood mononuclear cells, and anakinra treatment lead to normalization of disease-specific gene expression profile.[29,30]

Three inhibitors of IL-1 are currently available: anakinra, which is a recombinant IL-1 receptor antagonist, rilonacept, an IL-1 receptor fusion protein and canakinumab, a human IL-1 β antibody.


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