SHM Converge Scientific Abstract ePoster Gallery-When the Flame Ignites: A Case of Adult-Onset Still's Disease Complicated by Macrophage Activation Syndrome

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This case describes a 69-year-old man with prostate cancer undergoing radiation and androgen deprivation therapy who presented with hypotension, nausea, myalgias, and night sweats. On exam he had tachycardia, hypotension, and a maculopapular abdominal rash. Initial labs showed lactic acidosis, transaminitis, and pancytopenia, raising concern for sepsis, but the infectious evaluation was negative. He then developed fever and markedly elevated ferritin, prompting suspicion for hemophagocytic lymphohistiocytosis (HLH). When he later developed wrist pain, worsening rash, and recurrent fevers, clinicians considered adult-onset Still’s disease (AOSD), which can present with systemic inflammation and, in severe cases, cytopenias due to macrophage activation syndrome (MAS).<br /><br />He was ultimately diagnosed with AOSD complicated by MAS and improved after treatment with prednisone followed by a month-long taper. The report emphasizes that although AOSD classically causes neutrophilic leukocytosis, pancytopenia should alert clinicians to possible MAS or secondary HLH. MAS is a life-threatening hyperinflammatory syndrome marked by excessive activation of monocytes and macrophages, cytokine storm, high ferritin, cytopenias, liver dysfunction, and multiorgan involvement.<br /><br />The key message is the importance of early recognition of MAS in patients with suspected AOSD, especially when cytopenias replace the expected leukocytosis. Prompt diagnosis and targeted immunosuppressive therapy can significantly improve outcomes.

Asset Subtitle

Karl E. Herman

Meta Tag

Author List Karl E. Herman

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Macrophage Activation Syndrome

Concept Fever

Concept Cytopenia

Concept Hyperferritinemia

Distinguished Non-Finalist

Presenter Organization The Miriam Hospital

Presenting Author Karl E. Herman

Track Adult

Keywords

adult-onset Still's disease

macrophage activation syndrome

hemophagocytic lymphohistiocytosis

pancytopenia

ferritin

cytokine storm

systemic inflammation

prostate cancer

prednisone

hyperinflammatory syndrome

Adult-onset Stills disease

Macrophage Activation Syndrome

Fever

Cytopenia

Hyperferritinemia