Pediatric systemic lupus erythematosus with macrophage activation syndrome: navigating immune dysregulation under immunosuppressive therapy

Abstract

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can escalate into a more dangerous situation when accompanied by macrophage activation syndrome (MAS)—a rare but potentially deadly hyperinflammatory condition. This report explores the case of a 15-year-old female patient with an established history of systemic lupus erythematosus who developed MAS while on immunosuppressive therapy for chronic immune thrombocytopenic purpura. The patient presented with persistent high-grade fever, severe anemia, pancytopenia, and profound hyperferritinemia, yet notably lacked renal involvement—a feature that challenges conventional pediatric SLE presentations. Rapid deterioration necessitated aggressive treatment with high-dose corticosteroids, intravenous immunoglobulin, cyclosporine, and Rituximab, ultimately stabilizing her condition. This case underscores the diagnostic complexity and therapeutic challenges inherent in managing MAS in pediatric SLE, particularly when traditional markers are absent. This report offers insights into the timely diagnosis and treatment of rare MAS in SLE.