Intravenous administration of anakinra in children with macrophage activation syndrome

• Type of Material: Article
• Authors: Omkar Phadke, Emory University; Kelly Rouster Stevens, Emory University; Helen Giannopoulos, Childrens Healthcare Atlanta; Shanmuganathan Chandrakasan, Emory University; Sampath Prahalad, Emory University
• Language: English
• Date: 2021-06-29
• Publisher: BMC
• Publication Version: Final Published Version
• Copyright Statement: © The Author(s) 2021
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1186/s12969-021-00585-3
• Title of Journal or Parent Work: PEDIATRIC RHEUMATOLOGY
• Volume: 19
• Issue: 1
• Start Page: 98
• End Page: 98
• Grant/Funding Information: Dr. Prahalad is supported in part, by the Marcus Foundation Inc., Atlanta. No additional funding was secured for this study.
• Keywords: Rheumatology; Life Sciences & Biomedicine; JUVENILE IDIOPATHIC ARTHRITIS; PHASE-III; SEPSIS; Pediatrics; BLOCKADE; Science & Technology
• Research Categories: Health Sciences, Medicine and Surgery

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.