Macrophage activation syndrome (MAS), in its secondary form, often
complicates rheumatic diseases but rarely constitutes a mode of revelation.
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown
etiology that primarily affects women in adulthood. MAS is a serious condition
that may be the first presentation of SLE. Here, we report the case of a
4-year-old female with MAS as the primary manifestation of Systemic Lupus
Erythematosus (SLE). In this case, we outline the characteristics of a complex
case of SLE that was initially accompanied with MAS, and also review the
literature to discuss the clinical, biological, and therapeutic aspects of this
condition.
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