Abstract
7.3 Successful use of anakinra, a soluble IL-1 receptor antagonist, in pediatric rheumatic diseases associated macrophage activation syndrome/reactive hemophagocytic lymphohistiocytosis
Highlights
Increased interleukin 1 (IL-1) production characterizes macrophage activation syndrome/reactive hemophagocytic lymphohistiocytosis (MAS/rHLH), a potentially lethal complication of pediatric rheumatic diseases
All patients recovered from MAS/rHLH by 2 weeks; 5/6 pts discontinued corticosteroids by 5 weeks
Elevated baseline soluble IL-2 receptor (sIL2r) resolved by 48 hours post first dose of anakinra in all 3 patients tested
Summary
Increased interleukin 1 (IL-1) production characterizes macrophage activation syndrome/reactive hemophagocytic lymphohistiocytosis (MAS/rHLH), a potentially lethal complication of pediatric rheumatic diseases. Standard treatment (corticosteroids, cyclosporine, +/IVIg) is not always effective
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