%0 Case Reports %T Hemophagocytic Lymphohistiocytosis Unmasking Systemic Lupus Erythematosus: Management With Belimumab and a Case Study. %A Ahmad A %A Atluri R %A Robbins KJ %J Cureus %V 16 %N 7 %D 2024 Jul %M 39149687 暂无%R 10.7759/cureus.64596 %X Secondary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition caused by the hyperactivation of macrophages and T-cells, triggered by infection, malignancy, or underlying rheumatological conditions. It rarely presents as a first manifestation of a rheumatological condition. Macrophage activation syndrome (MAS) is secondary HLH associated with underlying hematological conditions. Here, we present a case of a previously healthy 29-year-old female who was admitted with fever, rash, and pancytopenia, found to have HLH, and a workup revealed underlying systemic lupus erythematosus (SLE). She was successfully treated with dexamethasone, etoposide, and belimumab, with complete recovery of her symptoms. This case highlights the importance of a thorough evaluation of rheumatological conditions in all patients with HLH despite their previous medical history and the use of belimumab for SLE.