Beyond the Flare: A Case of Systemic Lupus Erythematosus Complicated by Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal hyperinflammatory syndrome marked by excessive immune activation, cytokine storm, multiorgan failure, and high mortality. Systemic lupus erythematosus (SLE) is considered to be one of the triggers. SLE-associated HLH is especially challenging to diagnose and manage, as it can mimic lupus flares. We describe a 30‑year‑old woman with SLE who developed secondary HLH. Initially thought to have an SLE flare, she rapidly deteriorated with multiorgan dysfunction. The diagnosis of HLH was confirmed using the HLH-2004 criteria and a significantly elevated H-score. Despite intensive treatment with corticosteroids and immunosuppressive agents, the patient ultimately died from HLH-related complications. This case highlights the critical need for early recognition and prompt differentiation of HLH from lupus flares in SLE patients. Timely, targeted therapy is essential to improve outcomes in this high-risk group.