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

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A 20-year-old woman with no significant past medical history presented with fever, odynophagia, rash, tachycardia, and tender erythematous lesions on the hands, ankles, and face. Extensive infectious and autoimmune workup was negative, including blood cultures, respiratory and hepatitis panels, HIV, gonorrhea/chlamydia, mono, strep, Lyme, ANA, anti-dsDNA, anti-smooth muscle, and anti-Jo-1. Imaging showed cervical lymphadenopathy, but CT chest/abdomen/pelvis and bone marrow biopsy found no malignancy.  <br /><br />During her first admission, she had fever, leukocytosis, and elevated ferritin, and the initial impression was post-infectious immune activation after a recent viral illness. On readmission, recurrent fevers and rash were accompanied by markedly elevated inflammatory markers, including ferritin, triglycerides, IL-2, and CXCL9, leading to a diagnosis of hemophagocytic lymphohistiocytosis (HLH), and dexamethasone was started. After steroid tapering, symptoms recurred along with hepatitis. At that point, she met Yamaguchi criteria for adult-onset Still’s disease (AOSD), and the diagnosis was revised to AOSD complicated by macrophage activation syndrome (MAS), a form of secondary HLH. She improved and remained clinically stable after treatment with anakinra.  <br /><br />The case highlights that AOSD is a rare inflammatory disease that can be difficult to diagnose because its symptoms are nonspecific and it is a diagnosis of exclusion. It also emphasizes that MAS/HLH can be life-threatening and should be considered when fever, rash, hyperferritinemia, and systemic inflammation persist despite negative infectious and malignant evaluations.

Asset Subtitle

Andrew L. Wu

Meta Tag

Author List Andrew L. Wu

Category Clinical Vignettes

Concept Adult-onset Stills disease

Concept Macrophage Activation Syndrome

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Yamaguchi criteria

Concept HLH-04 criteria

Distinguished Non-Finalist

Presenter Organization University of Wisconsin

Presenting Author Andrew L. Wu

Track Adult

Keywords

adult-onset Still’s disease

macrophage activation syndrome

hemophagocytic lymphohistiocytosis

hyperferritinemia

fever

rash

inflammatory markers

anakinra

Yamaguchi criteria

secondary HLH

Adult-onset Stills disease

Macrophage Activation Syndrome

Secondary hemophagocytic lymphohistiocytosis

Yamaguchi criteria

HLH-04 criteria