SHM Converge Scientific Abstract ePoster Gallery-Early Recognition of Macrophage Activation Syndrome Secondary to Sjogren's Syndrome

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This case describes a 30-year-old previously healthy woman who presented with recurrent high fevers, nausea, rash, and dactylitis and was found to have severe inflammation with marked hyperferritinemia. Her evaluation included infectious, malignant, and rheumatologic workups, with consultations from Infectious Disease, Rheumatology, and Hematology. Infectious studies were negative, and CT imaging did not reveal a clear malignancy or other trigger. Autoimmune testing instead supported previously undiagnosed Sjögren’s syndrome and mixed connective tissue disease.<br /><br />Because she also had elevated transaminases and bicytopenia, clinicians suspected macrophage activation syndrome (MAS), a rare but potentially fatal hyperinflammatory syndrome that can progress to cytokine storm if not recognized promptly. She was started on dexamethasone 10 mg daily before all confirmatory send-out tests were available. Bone marrow biopsy later showed hemophagocytosis and hypocellular marrow, supporting the diagnosis. Soluble IL-2 receptor and natural killer cell testing were sent but not returned during hospitalization.<br /><br />After steroid treatment, her fever, rash, and fatigue improved significantly. She was discharged after a 7-day hospital stay on continued dexamethasone with close hematology and rheumatology follow-up.<br /><br />The presentation emphasizes that MAS is difficult to diagnose early because definitive criteria may rely on specialized tests that are not rapidly available. In adults, the HScore can help estimate likelihood quickly, while HLH-2004 and PRINTO criteria are also used but may be less practical in urgent settings. The report highlights the importance of early suspicion, multidisciplinary evaluation, and prompt treatment to reduce the high mortality associated with MAS.

Asset Subtitle

Michael Frerick

Meta Tag

Author List Jay Anderson, Michael Frerick

Category Clinical Vignettes

Concept Macrophage Activation Syndrome

Concept Fever

Concept Cytopenia

Concept Hyperferritinemia

Concept Corticosteroid

Distinguished Non-Finalist

Presenter Organization OhioHealth

Presenting Author Michael Frerick

Track Adult

Keywords

macrophage activation syndrome

hyperferritinemia

Sjögren’s syndrome

mixed connective tissue disease

cytokine storm

hemophagocytosis

dexamethasone

bicytopenia

HScore

rheumatology

Macrophage Activation Syndrome

Fever

Cytopenia

Hyperferritinemia

Corticosteroid