Abstract
BackgroundSubcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis. Recent reports suggest anakinra can be a valuable addition to the treatment of COVID-19 associated cytokine storm syndrome and the related multisystem inflammatory syndrome (MIS-C) in children. Herein, we describe our experience with intravenously administered anakinra.Findings19 Patients (9 male) received intravenous (IV) anakinra for treatment of macrophage activation syndrome (MAS) secondary to systemic lupus erythematosus (SLE), systemic JIA (SJIA) or secondary hemophagocytic lymphohistiocytosis (sHLH). In most cases the general trend of the fibrinogen, ferritin, AST, and platelet count (Ravelli criteria) improved after initiation of IV anakinra. There were no reports of anaphylaxis or reactions associated with administration of IV anakinra.ConclusionIntravenous administration of anakinra is an important therapeutic option for critically ill patients with MAS/HLH. It is also beneficial for those with thrombocytopenia, subcutaneous edema, neurological dysfunction, or very young, hospitalized patients who need multiple painful subcutaneous injections.
Highlights
Subcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis
Anakinra has been described to be effective in the treatment of macrophage activation syndrome (MAS) secondary to sJIA as well as other rheumatic diseases like systemic lupus erythematosus (SLE) and Kawasaki Disease (KD) [2,3,4,5]
Recent reports show anakinra can be effective in secondary hemophagocytic lymphohistiocytosis due to non-rheumatic diseases as well [6]
Summary
Subcutaneous anakinra is an interleukin-1 inhibitor used to treat juvenile idiopathic arthritis. Anakinra has been described to be effective in the treatment of macrophage activation syndrome (MAS) secondary to sJIA as well as other rheumatic diseases like systemic lupus erythematosus (SLE) and Kawasaki Disease (KD) [2,3,4,5]. Recent reports show anakinra can be effective in secondary hemophagocytic lymphohistiocytosis (sHLH) due to non-rheumatic diseases as well [6]. In some situations, such as thrombocytopenia, subcutaneous edema or in children in intensive care setting, it may be necessary to use intravenous (IV) administration of anakinra instead of SC anakinra. We sought to describe our experience with IV anakinra in children with MAS at our institution prior to COVID-19 in order to guide clinicians wishing to consider this therapy for indications such as hyperinflammation seen with
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