Journal of Nepal Medical Association (Nov 2021)
Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report
Abstract
Macrophage activation syndrome is a rare but a life threatening condition commonly associated with Systemic Juvenile Idiopathic Arthritis. Its clinical presentation includes fever, hepatosplenomegaly, hypertriglyceridemia, hypofibrinogenemia, hyperferritinemia and impaired liver enzymes. The symptoms are alarming yet non-specific and often lead to a delayed diagnosis. A 12 year male presented with a history of intermittent fever and was started on antibiotics but failed to respond after several days of hospital stay. After a series of investigations to rule out multiple diagnoses he was diagnosed as a case of Macrophage Activation Syndrome secondary to Systemic onset Juvenile Arthritis and was treated with steroids.
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