SHM Converge Scientific Abstract ePoster Gallery-Macrophage Activation Syndrome: A Potential Consequence of Adult-Onset Still's Disease

Pdf Summary

This case describes a 24-year-old woman with adult-onset Still’s disease (AOSD), an inflammatory disorder marked by fever, arthralgias, rash, and very high ferritin. She presented with a month of intermittent non-pruritic rash, migratory joint pain, fevers, pharyngitis, and lymphadenopathy. Her symptoms began after travel to Mexico and temporarily improved with a short steroid course, but later recurred with worsening systemic symptoms.<br /><br />On admission, she was febrile to 39.3°C and tachycardic. Laboratory testing showed leukocytosis, elevated inflammatory markers, negative ANA and RF, and striking hyperferritinemia to 29,972. Infectious Disease was consulted, and an extensive infectious workup was started but was initially negative. As her course progressed, liver enzymes rose and her red blood cells, white blood cells, and platelets began to fall, raising concern for macrophage activation syndrome (MAS), a dangerous complication of AOSD. Hematology was consulted, and she improved after treatment with high-dose IV steroids.<br /><br />The discussion emphasizes that AOSD can be difficult to diagnose because symptoms are nonspecific and overlap with infection, malignancy, and other rheumatologic diseases. Maintaining a high clinical suspicion is important to avoid delayed treatment and to recognize MAS early. This is especially important because AOSD-associated MAS carries a significant mortality risk, reported at 10–20%.<br /><br />The presentation also reviews the Yamaguchi criteria for AOSD diagnosis, which require five total criteria, including at least two major criteria, and exclusion of infection, malignancy, and other rheumatic disease.

Asset Subtitle

Cecelia Hale

Meta Tag

Author List Cecelia Hale, Kevin Kinduell, Nang Lin, Sampath Ethiraj

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Macrophage Activation Syndrome

Concept Fever

Concept Hyperferritinemia

Concept High-Dose Corticosteroid

Distinguished Non-Finalist

Presenter Organization Parkview Health

Presenting Author Cecelia Hale

Track Adult

Keywords

adult-onset Still's disease

AOSD

macrophage activation syndrome

hyperferritinemia

fever

arthralgia

rash

pharyngitis

lymphadenopathy

Yamaguchi criteria

Adult-onset Stills disease

Macrophage Activation Syndrome

Fever

Hyperferritinemia

High-Dose Corticosteroid