Macrophage Activation Syndrome/Secondary Hemophagocytic Lymphohistiocytosis in Adult-Onset Still's Disease: An Uncommon Initial Presentation in a Young Nepalese Female: A Case Report

    January 2025 in “ Clinical Case Reports ”
    Sunil Bogati, Kajal Bishwakarma, Sunil Bogati, Saket Jha, Sunil Bogati, Saket Jha
    TLDR Timely and aggressive treatment is crucial for improving outcomes in severe complications of adult-onset Still's disease.
    This case report discusses a 36-year-old Nepalese female with adult-onset Still's disease (AOSD) who presented with symptoms including fever, joint pain, rash, hair loss, and weight loss, and was diagnosed with macrophage activation syndrome (MAS), a severe complication of AOSD. Despite initial treatment with IV steroids, her condition required further intervention with dexamethasone and cyclosporine, which led to improvement. The report highlights the critical nature of MAS, which occurs in about 15% of AOSD patients and has a mortality rate over 50%, emphasizing the importance of timely and aggressive treatment.
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