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Macrophage Activation Syndrome in two girls with Systemic Lupus Erythematosus

N. Iagaru, D. Oraseanu, F. Rusu, Monica Luminos, Anca Draganescu, Anca Ghit
Abstract: Background: Most frequently seen in Systemic Arthritis - Juvenile Idiopathic Arthritis, macrophage activation syndrome (MAS) may also be a life-threatening complication in juvenile systemic lupus erythematosus (SLE). The diagnosis of MAS may be particularly challenging because it may mimic the clinical and laboratory features of the underlying disease. Objective: To describe the clinical and laboratory features of MAS as an early complication of juvenile systemic lupus erythematosus. Methods: We report two cases of females with MAS in Juvenile SLE occurring acutely in the first 6 months after the onset, at the time of first presentation in our hospital. The clinical features and laboratory data were analyzed. The main laboratory findings of MAS were present: pancytopenia, abnormal serum hepatic enzyme levels, coagulopathy, neurologic symptoms, hyperferritinemia, hypertriglyceridemia, decreased erythrocyte sedimentation rate, hyponatremia, hypoalbuminemia and macrophage hemophagocytosis in the bone marrow aspirate sample. The treatment included intravenous methylprednisolon, immunoglobulins, cyclophosphamide pulse (one case), transfusions (PT, FFP, PRC) and supportive therapy. Conclusion: The diagnosis of MAS can be difficult because some of its clinical features overlap those of lupus itself. The occurrence of unexplained fever and pancytopenia associated with increased hepatic enzyme levels, coagulopathy, hyperferritinemia should promptly raise the suspicion of macrophage activation syndrome.

Keywords: MAS, SLE, children


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