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 rare initial presentation of macrophage activation syndrome (MAS) in a 36-year-old Nepalese female with adult-onset Still's disease (AOSD). MAS is a life-threatening complication of AOSD, characterized by symptoms such as fever, joint pain, rash, and extremely high serum ferritin levels. The patient was diagnosed with AOSD and MAS after ruling out infections and other autoimmune diseases. Treatment involved high-dose steroids, which initially improved her condition, but further complications required additional immunomodulatory therapy. The report emphasizes the importance of early recognition and aggressive treatment of MAS in AOSD patients, as it significantly increases mortality risk.
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