A Case of Macrophage Activation Syndrome During the Treatment of Adult-onset Still's Disease With Tocilizumabopen accessA Case of Macrophage Activation Syndrome During the Treatment of Adult-onset Still's Disease With Tocilizumab
- Other Titles
- A Case of Macrophage Activation Syndrome During the Treatment of Adult-onset Still's Disease With Tocilizumab
- Authors
- 이주호; 하유정; 강은하; 장성혜; 이윤종
- Issue Date
- Apr-2022
- Publisher
- 대한류마티스학회
- Keywords
- Still’ s disease; adult-onset; Macrophage activation syndrome; Lymphohistiocytosis; hemophagocytic; Tocilizumab
- Citation
- 대한류마티스학회지, v.29, no.2, pp 123 - 128
- Pages
- 6
- Journal Title
- 대한류마티스학회지
- Volume
- 29
- Number
- 2
- Start Page
- 123
- End Page
- 128
- URI
- https://scholarworks.bwise.kr/sch/handle/2021.sw.sch/20731
- DOI
- 10.4078/jrd.2022.29.2.123
- ISSN
- 2093-940X
2233-4718
- Abstract
- Macrophage activation syndrome (MAS) is a fatal complication of adult-onset Still’s disease (AOSD). Although anti-cytokine agents have been recommended for refractory AOSD or complicated with MAS, MAS cases have been rarely reported during anticytokine treatment. Herein, we describe the first AOSD case complicated with MAS during the treatment with tocilizumab in Korea. Two years after tocilizumab maintenance therapy, high fever and hypertransaminasemia recurred. MAS was diagnosed based on hyperferritinemia, elevated soluble IL-2 receptor levels, and the presence of hemophagocytic histiocytes in the bone marrow. However, she had normal white blood cell counts and acute phase reactant levels. High-dose glucocorticoid and anakinra therapies were not effective, but her disease improved with etoposide. This case shows that tocilizumab may not prevent MAS development and can modify clinical features making it challenging to diagnose. Cytotoxic therapy such as etoposide may be required in MAS cases that develop during anti-cytokine therapy.
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