SHM Converge Scientific Abstract ePoster Gallery-Refractory Macrophage Activation Syndrome Associated With Adult-Onset Still's Disease

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This case report describes refractory macrophage activation syndrome (MAS) in a 29-year-old woman with adult-onset Still’s disease (AOSD). MAS is a severe hyperinflammatory complication of rheumatic disease that can resemble an AOSD flare, making diagnosis and treatment difficult.<br /><br />The patient initially presented with features consistent with both AOSD and MAS, including polyarthralgia, rash, high fevers, hepatosplenomegaly, lymphadenopathy, leukocytosis, elevated AST, hypertriglyceridemia, very high ferritin, and elevated soluble CD25. Infectious, autoimmune, and malignancy evaluations were negative. Although a lymph node biopsy was nonspecific and the first bone marrow biopsy showed no hemophagocytosis, clinicians suspected MAS and treated her with anakinra and prednisone, leading to temporary improvement.<br /><br />Two weeks later, she returned with worsening dyspnea. She again met clinical criteria for AOSD and MAS, but now had pancytopenia and new systolic heart failure with an ejection fraction of 25–30%, suggesting autoinflammatory cardiomyopathy. A repeat bone marrow biopsy then demonstrated hemophagocytosis. Because of persistent clinical concern for refractory MAS, etoposide was started in addition to glucocorticoids, anakinra, and heart failure therapy. Her pancytopenia and cardiac symptoms improved afterward.<br /><br />The authors emphasize that MAS and AOSD share many clinical and inflammatory features, but MAS can require escalation to MAS-specific treatment even when biopsy findings are absent or nonspecific. They conclude that if a patient persistently meets clinical criteria for MAS, treatment should be intensified based on the clinical picture rather than relying solely on histopathology.

Asset Subtitle

Subhan Tabba

Meta Tag

Author List Subhan Tabba, Tapan Patel, William T. Farrington

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Macrophage Activation Syndrome

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Yamaguchi criteria

Concept H-score

Distinguished Non-Finalist

Presenter Organization Texas Health Presbyterian Hospital of Dallas

Presenting Author Subhan Tabba

Track Adult

Keywords

macrophage activation syndrome

adult-onset Still’s disease

refractory MAS

hyperinflammatory syndrome

hemophagocytosis

anakinra

etoposide

pancytopenia

autoinflammatory cardiomyopathy

systolic heart failure

Adult-onset Stills disease

Macrophage Activation Syndrome

Secondary hemophagocytic lymphohistiocytosis

Yamaguchi criteria

H-score