Management Challenge of Coexistence of Macrophage Activation Syndrome, Systemic Lupus Erythematosus, and Hepatitis B: Case Report

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal clinical and histological entity. Macrophage Activation Syndrome (MAS) is an HLH secondary to rheumatic and autoimmune diseases. Secondary MAS in systemic lupus erythematosus (SLE) is underdiagnosed. Its treatment is not yet standardized. The treatment of MAS in the context of SLE primarily involves corticosteroids and immunosuppressants, with the potential addition of Intravenous Immunoglobulins (IVIG) and biological treatments for refractory cases. Early detection and prompt intervention are crucial to reduce associated mortality. The presence of infection worsens the patient's prognosis. The coexistence of SLE and hepatitis B during MAS is rarely described in the literature, and its management remains debated. We report the case of a 42-year-old Malagasy woman presenting with SLE complicated by MAS at the time of diagnosis, associated with viral hepatitis B.