SHM Converge Scientific Abstract ePoster Gallery-He's Still Standing: A Battle With Macrophage Activation Syndrome

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This case describes a 38-year-old man with a history of presumed seronegative rheumatoid arthritis who presented with dyspnea, pleuritic chest pain, and fever, after a year of arthralgias, recurrent sore throat, and relapsing fevers. Initial labs showed marked inflammation, including leukocytosis, elevated creatinine, CRP, ESR, and ferritin, with negative ANA and RF. Despite broad-spectrum antibiotics, he worsened and developed acute hypoxic respiratory failure with mediastinitis, pneumonia, pleural effusions, and pericarditis with a large pericardial effusion.<br /><br />Extensive infectious and rheumatologic evaluations were unrevealing. He initially improved with steroids, but fever and a morbilliform rash returned during tapering, raising suspicion for adult-onset Still’s disease (AOSD), even though the presentation was atypical. The discussion highlights that AOSD should be considered in patients with unexplained fever, inflammatory rash, arthralgias, and serositis, and that the Yamaguchi criteria are useful for diagnosis.<br /><br />The case also emphasizes recognizing macrophage activation syndrome (MAS), a life-threatening complication of AOSD. In this patient, worsening fevers, pleural and pericardial effusions increased concern for MAS. Bone marrow biopsy was negative for hemophagocytic lymphohistiocytosis, and cervical lymph node biopsy supported an inflammatory rheumatic process. Ultimately, high-dose methylprednisolone and anakinra controlled his symptoms and improved inflammatory markers, confirming AOSD complicated by peripheral MAS. He remained well controlled on anakinra 18 months later.

Asset Subtitle

Jacqueline Lippert

Meta Tag

Author List Adrianna L. Elashker, Jacqueline Lippert, Suma Menon

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Macrophage Activation Syndrome

Concept Yamaguchi criteria

Concept Fever

Concept Arthralgia

Distinguished Non-Finalist

Presenter Organization Atrium Health Wake Forest Baptist

Presenting Author Jacqueline Lippert

Track Adult

Keywords

adult-onset Still’s disease

macrophage activation syndrome

seronegative rheumatoid arthritis

pleuritic chest pain

pericardial effusion

pleural effusions

morbilliform rash

Yamaguchi criteria

anakinra

high-dose methylprednisolone

Adult-onset Stills disease

Macrophage Activation Syndrome

Yamaguchi criteria

Fever

Arthralgia