Adult-onset Still's disease complicated with hemophagocytic syndrome

Journal of the Formosan Medical Association = Taiwan Yi Zhi
Horng-Ming YehChang-Hung Chen

Abstract

Hemophagocytic syndrome (HS) that occurs in the course of adult-onset Stills disease (AOSD) has been reported only rarely in the literature. HS and AOSD share overlapping clinical and laboratory features, therefore, it is difficult to recognize HS as a complication of AOSD. Here, we report the case of a 46-year old woman with classical features of AOSD. Severe pancytopenia and jaundice associated with extreme hyperferritinemia occurred during high-dose steroid treatment. Bone marrow biopsy showed typical pathological features of hemophagocytosis, which confirmed the coexistence of HS with AOSD. The patient was treated with methylprednisolone pulse therapy of 500 mg/day for 3 days, as recommended in cases of HS complicating AOSD, and her condition improved gradually. During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine.

Citations

Oct 15, 2013·International Journal of Rheumatic Diseases·Rathindra N SarkarOmar S Mullick
Jun 29, 2011·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Arwa MekiJim Murray
Apr 14, 2018·Modern Rheumatology·Toshihide MimuraTakayuki Sumida
Jun 17, 2020·Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases·Samer Al-HumadiVelimir A Luketic

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Adult-onset Still's disease (AOSD) is a form of Still's disease, a rare systemic autoinflammatory disease characterized by the classic triad of persistent high spiking fevers, joint pain, and a distinctive salmon-colored bumpy rash. Discover the latest research on AOSD here.