Macrophage activation syndrome at the onset of glucocorticoid-resistant systemic lupus erythematosus: a case report

Abstract

Introduction. Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory state mediated by uncontrolled cytokine storm and haemophagocytosis. Although rarely reported, MAS might occur in systemic lupus erythematosus (SLE), notably as an inaugural manifestation. Glucocorticoids (GCs) are the cornerstone of SLE therapy. However, in some cases high doses of GCs are required to achieve remission (i.e. glucocorticoid-resistance), leading to significant side effects.

Keywords

• macrophage activation syndrome
• systemic lupus erythematosus
• hyperferritinemia
• glucocorticoid-resistance
• high-dose corticotherapy