Annals of Internal Medicine: Clinical Cases · Published 2026-08-01 · DOI 10.7326/aimcc.2025.1207
Marie-Anne Heusler, Dominik Heim, Christine Bernsmeier, Diego Kyburz, Clemens Winterhalder, Marten Trendelenburg
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening systemic inflammatory syndrome associated with hemophagocytosis in the reticuloendothelial system. Secondary or acquired forms can occur inter alia in the context of autoimmune/inflammatory diseases. Approximately 1% to 5% of patients with systemic lupus erythematosus are affected, although typically later in the course of the disease. Here, we report the uncommon case of a patient presenting with developing HLH at the initial diagnosis of systemic lupus erythematosus. Developing HLH was diagnosed in the context of elevated cholestatic liver enzymes, intrasinusoidal erythrophagocytosis on liver biopsy alongside fever, hypertriglyceridemia, hyperferritinemia, pancytopenia, and elevated soluble interleukin-2 receptor. Immunosuppressive treatment led to clinical and biochemical resolution.
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Heusler, M., Heim, D., Bernsmeier, C., et al. (2026). Systemic Lupus Erythematosus Complicated by Hemophagocytic Lymphohistiocytosis Presenting as Cholestatic Liver Disease. Annals of Internal Medicine: Clinical Cases. https://doi.org/10.7326/aimcc.2025.1207