Macrophage Activation Syndrome: Calming the Cytokine Storm

This talk from pediatric rheumatologist Daniel Soulsby, MD, lays out helpful ways to think about macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH), conditions that are related, share many signs (including hyperferritinemia, cyclic fevers and cytopenias) and can be fatal. He discusses systemic juvenile idiopathic arthritis, which may set the stage for MAS; how to work up patients with symptoms of hyperinflammation; and research showing the potentially lifesaving value of using high doses of the rheumatoid arthritis drug anakinra.

Learning Objectives:

• Identify rheumatic diseases associated with the development of macrophage activation syndrome
• Evaluate the similarities and differences between macrophage activation syndrome (MAS) and secondary vs primary hemophagocytic lymphohistiocytosis (HLH)
• Use current diagnostic workup and treatment approaches for macrophage activation syndrome (MAS)