Uncommon Case of Hemophagocytic Lymphohistiocytosis as the First Presentation of Systemic Lupus Erythematosus

Hemophagocytic lymphohistiocytosis secondary to systemic lupus erythematosus (SLE) is a potentially fatal condition that is frequently misdiagnosed, particularly when its occurrence is the first manifestation of SLE. Diagnosis is often delayed due to overlapping clinical and laboratory findings with infections and autoimmune diseases. We report a case of a young, healthy woman with hemophagocytic lymphohistiocytosis as the first manifestation of SLE. Early multispecialist involvement, comprehensive laboratory testing, and liver biopsy led to prompt diagnosis. Treatment with glucocorticoids, anakinra, and hydroxychloroquine led to rapid clinical and laboratory improvement. This case illustrates how collaboration and prompt diagnosis and treatment can improve survival of patients with this life-threatening condition.