SHM Converge Scientific Abstract ePoster Gallery-A Case of Adult-Onset Still's Disease Initially Difficult to Distinguish From Drug Eruption and Nonspecific Rash

Pdf Summary

This case describes a 75-year-old woman with persistent fever, diffuse rash, and markedly abnormal liver enzymes initially thought to be due to biliary disease, infection, or a drug eruption after ceftriaxone. Imaging suggested gallbladder wall thickening and gallstones, but endoscopy found no biliary obstruction. Her inflammatory markers were elevated, especially ferritin (27,428 ng/mL) and soluble IL-2 receptor, raising concern for hyperinflammation such as hemophagocytic syndrome. Extensive infectious and autoimmune testing was negative, though bone marrow biopsy showed hemophagocytosis.<br /><br />She was treated with methylprednisolone pulse therapy, but fever and rash recurred when steroids were tapered, indicating steroid-refractory disease. Cyclosporine A was added, after which the team re-evaluated her symptoms. She later developed sore throat and cough and ultimately met Yamaguchi’s criteria for adult-onset Still’s disease (AOSD). The final diagnosis was AOSD complicated by hemophagocytosis/macrophage activation syndrome.<br /><br />The report emphasizes that in older adults, AOSD can be difficult to recognize because rash may be atypical and can resemble drug eruptions or other hyperinflammatory rashes. Persistent quotidian fever, recurrent rash, negative infectious workup, very high ferritin, and hemophagocytosis should prompt consideration of AOSD. Early immunosuppressive treatment beyond glucocorticoids, including cyclosporine, may be needed when macrophage activation is present or symptoms recur during steroid tapering.

Asset Subtitle

Natsumi Omoto

Meta Tag

Author List Eri Sumii, Masaya Kato, Naoya Matsuhara, Natsumi Omoto, Ryutaro Masaki, Saki Aihara, Syunsuke Yorifuji, Takayuki Hoso, Tatsuki Fujii, Wakako Harada, Yutaro Yuzuriha, Yuya Shigenobu

Category Clinical Vignettes

Concept Adult-Onset Still Disease

Concept Fever

Concept Secondary hemophagocytic lymphohistiocytosis

Concept Hemophagocytosis

Concept Corticosteroid

Distinguished Non-Finalist

Presenter Organization Hiroshima City North Medical Center Asa Citizens Hospital General Medicine

Presenting Author Natsumi Omoto

Track Adult

Keywords

adult-onset Still's disease

AOSD

macrophage activation syndrome

hemophagocytic syndrome

persistent fever

diffuse rash

hyperferritinemia

bone marrow hemophagocytosis

cyclosporine A

steroid-refractory inflammation

Adult-Onset Still Disease

Fever

Secondary hemophagocytic lymphohistiocytosis

Hemophagocytosis

Corticosteroid