Intravenous administration of anakinra in children with macrophage activation syndrome

Pediatr Rheumatol Online J. 2021 Jun 29;19(1):98. doi: 10.1186/s12969-021-00585-3.

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.

MeSH terms

  • Administration, Intravenous / methods*
  • Antirheumatic Agents / administration & dosage
  • COVID-19 / epidemiology
  • COVID-19 / immunology
  • COVID-19 Drug Treatment*
  • Child
  • Child, Hospitalized / psychology
  • Critical Illness / psychology
  • Critical Illness / therapy
  • Cytokine Release Syndrome / drug therapy
  • Cytokine Release Syndrome / virology
  • Female
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / administration & dosage*
  • Lupus Erythematosus, Systemic* / complications
  • Lupus Erythematosus, Systemic* / immunology
  • Lupus Erythematosus, Systemic* / therapy
  • Lymphohistiocytosis, Hemophagocytic* / complications
  • Lymphohistiocytosis, Hemophagocytic* / immunology
  • Lymphohistiocytosis, Hemophagocytic* / therapy
  • Macrophage Activation Syndrome* / blood
  • Macrophage Activation Syndrome* / drug therapy
  • Macrophage Activation Syndrome* / etiology
  • Male
  • Patient Comfort / methods
  • Retrospective Studies
  • SARS-CoV-2
  • Systemic Inflammatory Response Syndrome / drug therapy*

Substances

  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein

Supplementary concepts

  • pediatric multisystem inflammatory disease, COVID-19 related