Abstract Systemic lupus erythematosus (SLE) can rarely present with hemophagocytic lymphohistiocytosis (HLH), a severe hyperinflammatory state with high mortality if unrecognized. We report a 21-year-old male who presented with fever, ascites, pleural effusion, anemia, and acute kidney injury. Infectious causes were ruled out, and autoimmune markers confirmed SLE. Bone marrow biopsy showed hemophagocytosis, fulfilling HLH-2004 criteria for HLH. The patient responded well to corticosteroids and hydroxychloroquine, with plans for cyclophosphamide and renal biopsy. This case emphasizes the importance of early recognition of macrophage activation syndrome in SLE patients with systemic inflammation and cytopenias.
Shende et al. (Wed,) studied this question.