Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra
Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra
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DOI：
10.1111/imj.12002
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发表时间：
2012-12-01
影响因子：
2.1
通讯作者：
Prentice, D.
中科院分区：
文献类型：
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作者：
Loh, N. K.;Lucas, M.;Prentice, D.
一位先前健康的20岁男性出现成人斯蒂尔病(ASD)。他患上了威胁生命的巨噬细胞激活综合征(MAS)，对标准免疫抑制无效，但对IL-1受体拮抗剂Anakinra有显著反应。随后的免疫学检查包括评估自然杀伤(NK)细胞和CD8+T细胞的穿孔素表达，这证实了MAS。
A previously healthy 20-year-old man presented with adult Still disease (ASD). He developed life-threatening macrophage activation syndrome (MAS), which was refractory to standard immunosuppression but responded dramatically to the IL-1 receptor antagonist anakinra. Subsequent immunological investigations included assessment of the perforin expression of natural killer (NK) cells and CD8+ T cells, which confirmed MAS.