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Macrophage activation syndrome (MAS) is defined as a specific secondary hemophagocytic lymphohistiocytosis that refers particularly to those triggered by autoimmune diseases. MAS is a rare and highly lethal complication of systemic lupus erythematosus (SLE), which can be associated with, or mimic, disease flare. However, the data regarding the clinical course, management and outcome of SLE with MAS is limited, especially in adults. Lack of clinical recognition of the disease often leads to poor prognosis.

Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus: A case report