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

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 sympto...

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Autores principales: Rishikesh Kafle, Anwesh Bhatta, Sumit Gami, Abhin Sapkota, Dipesh Sharma, Arabindra Yadav, Vijaya Kumar Chikanbanjar
Formato: article
Lenguaje:EN
Publicado: Nepal Medical Association 2021
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Acceso en línea:https://doaj.org/article/5d5177583b094c38bac25c8390c4ecc8
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Sumario: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.