A variant of hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome (MAS) is seen in autoimmune diseases such as systemic lupus erythematosus (SLE), Systemic onset juvenile idiopathic arthritis. It is known to present as a complication in the course of these autoimmune conditions. However, the occurrence of MAS as the first presentation of SLE, can pose a challenge in its timely diagnosis and management in account of the similarity in their clinical presentation. Herein we repo
