A Case of Macrophage Activation Syndrome Manifesting as the Initial Presentation of Systemic Lupus Erythematosus

Macrophage activation syndrome (MAS) is a potentially fatal complication of an autoimmune rheumatologic disease characterized by overwhelming inflammation, multiorgan failure, and high mortality if untreated. We report a rare case of a 56-year-old man who presented with fever for three weeks and had a constellation of clinical features and laboratory findings, meeting the diagnostic criteria for systemic lupus erythematosus (SLE) and SLE-associated MAS. He was treated with high dose intravenous corticosteroid and hydroxychloroquine, resulting in resolution of fever and dramatic clinical improvement.