Systemic Lupus Erythematosus Presenting as Macrophage Activation Syndrome

Macrophage Activation Syndrome (MAS), a potentially life threatening condition belongs to acquired cause of Hemophagocytic lymphohistiocytosis (HLH) group of diseases more often found in children than adults. It presents as familial forms or acquired forms that include infectious aetiologies notably viral, malignancies, drugs and autoimmune diseases. Its association with several systemic autoimmune disease includes commonly systemic Juvenile Idiopathic Arthritis (sJIA), adult-onset Still's disease and rarely with Systemic Lupus Erythematosus (SLE), rheumatoid arthritis and dermatomyositis. It is a heterogeneous presentation of systemic and laboratory findings characterised by abnormal immune activation and inflammation warrants early diagnosis and prompt treatment. Herein we report a rare case of 15-year-old female who presented with fever of two months duration, who was later diagnosed to be having MAS with SLE. Despite the rarity MAS can be initial presenter in SLE and should be searched in the background of fever and features of SLE. Initiation of treatment with pulse methylprednisolone and follow up with oral hydroxychloroquine, cyclosporine and steroids had a favourable outcome.