Immunomodulation in sepsis-induced macrophage activation syndrome in children

Abstract

Background: Sepsis is a state of systemic inflammation due to an infectious etiology that may lead to multisystem dysfunction, hemodynamic instability, and even death. It has been postulated that there may be an underlying immunomodulatory process resulting from rapid and exaggerated activation of macrophages that results in a cytokine storm and the development of macrophage activation syndrome (MAS). Adding immunomodulation to standard therapy (antibiotics and supportive care) can improve the prognosis. Clinical Description: We present a series of three young children who presented with the clinical features of sepsis. All three showed poor clinical response to management with timely antibiotics and supportive care, even after 48–72 h of initiation. In addition, there was the development of thrombocytopenia and transaminitis. The suspicion of MAS prompted us to order ferritin, triglyceride, and fibrinogen levels and applies the 2016 diagnostic criteria for MAS. These were satisfied, thus establishing the diagnosis. Management: In all three cases, immunomodulatory agents (intravenous immunoglobulin with or without pulses of methylprednisolone) were added, after which there was a clinical improvement, normalization of biomarkers, and complete recovery. Conclusion: Early immunomodulatory therapy, in addition to antibiotics, is beneficial in the successful treatment of children presenting with sepsis-induced MAS, thus preventing further morbidity and mortality and improving outcomes.

Keywords

• immunomodulation
• macrophage activation syndrome
• secondary hemophagocytic lymphohistiocytosis
• sepsis