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Intravenous administration of anakinra in children with macrophage activation syndrome

Omkar Phadke, Kelly Rouster‐Stevens, Helen T. Giannopoulos, Shanmuganathan Chandrakasan, Sampath Prahalad

2021Pediatric Rheumatology68 citationsDOIOpen Access PDF

Abstract

BACKGROUND: Subcutaneous 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. FINDINGS: 19 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. CONCLUSION: Intravenous 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.

Topics & Concepts

AnakinraMedicineMacrophage activation syndromeHemophagocytic lymphohistiocytosisRheumatologyInternal medicineTocilizumabGastroenterologyImmunologyArthritisDiseaseAutoimmune and Inflammatory Disorders ResearchHemophilia Treatment and ResearchOtitis Media and Relapsing Polychondritis
Intravenous administration of anakinra in children with macrophage activation syndrome | Litcius