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

Cureus. 2022 Jun 27;14(6):e26375. doi: 10.7759/cureus.26375. eCollection 2022 Jun.


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.

Keywords: corticosteroid; cytokine release storm; hemophagocytic lymphohistiocytosis (hlh); macrophage activation syndrome (mas); major hyperferritinemia; systemic lupus erythematosis.

Publication types

  • Case Reports