Macrophage Activation Syndrome in a Patient with Systemic Lupus Erythematosus

In 2019, a case study was conducted on a 37-year-old woman who presented with a 6-week history of a generalized nonpruritic erythematous rash, intermittent fever, weight loss of 5 kg, and diffuse hair loss. The patient was diagnosed with Macrophage Activation Syndrome (MAS) associated with Systemic Lupus Erythematosus (SLE). MAS is a hyperinflammatory syndrome characterized by excessive immune activation, leading to a cytokine storm, hemophagocytosis, and multiorgan damage. It is a life-threatening condition if not promptly identified and treated. The patient was treated with 500 mg methylprednisolone 3 times daily for the first 3 days and intravenous immunoglobulins at a dose of 1 g/kg for 2 consecutive days. After 14 days, she was started on mycophenolate mofetil 500 mg daily for 2 weeks, titrated up to 1 g twice daily, and steroids were slowly tapered. She responded clinically with resolution of intermittent fever and improvement of biochemical parameters and rash. The study concluded that clinicians should have a high degree of suspicion of MAS in a patient who is systemically unwell with pyrexia of unknown origin, cytopenias, and high ferritin levels.