Etanercept를 포함한 스테로이드, Cyclosporine A 병용요법으로 호전된 대식세포활성 증후군 1예

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neuro-logic symptoms. T...

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Bibliographic Details
Published in:Journal of rheumatic diseases 2012, 19(4), , pp.225-229
Main Authors: 심영석, Young Seok Shim, 김현수, Hyun Soo Kim, 김광남, Kwang Nam Kim
Format: Article
Language:Korean
Subjects:
Etanercept
Macrophage activation syndrome
MAS
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Summary:Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of childhood systemic inflammatory disorder, primarily systemic onset juvenile rheumatoid arthritis (SoJRA). It is characterized by pancytopenia, liver insufficiency, coagulopathy, and neuro-logic symptoms. The clinical manifestations are caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to cytokine overproduction including tumor necrosis factor-α (TNF-α). Methylpre-dnisolone pulse therapy and cyclosporine A have made a considerable progress in the treatment of MAS. However, the mortality rate remains high suggesting the need of another therapeutic agent. Several cases of MAS successfully treated with TNF-α inhibitor (etanercept) have been reported. We report the first Korean case of MAS successfully treated with combination therapy of corticosteroid, cyclosporine A and etanercept.
ISSN:2093-940X
2233-4718