• JNMA J Nepal Med Assoc · Nov 2021

    Case Reports

    Macrophage Activation Syndrome secondary to Systemic Juvenile Idiopathic Arthritis: A Case Report.

    • Rishikesh Kafle, Anwesh Bhatta, Sumit Gami, Abhin Sapkota, Dipesh Sharma, Arabindra Yadav, and Vijaya Kumar Chikanbanjar.
    • Department of Pediatrics, Kathmandu Medical College Teaching Hospital, Sinamangal, Kathmandu, Nepal.
    • JNMA J Nepal Med Assoc. 2021 Nov 15; 59 (243): 1166-1169.

    AbstractMacrophage 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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