致命性 NLRC4 相关过度炎症经 IL-18 抑制治疗成功。
Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition.
机构信息
Intramural Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, Md.
Division of Rheumatology, Department of Internal Medicine Specialties, University Hospitals of Geneva, Geneva, Switzerland.
出版信息
J Allergy Clin Immunol. 2017 May;139(5):1698-1701. doi: 10.1016/j.jaci.2016.10.022. Epub 2016 Nov 19.
Abstract
NLRC4-inflammasome hyperactivity causes infantile-onset Macrophage Activation Syndrome and enterocolitis with extraordinary serum IL-18 elevation (NLRC4-MAS). Herein, we report a critically ill infant with severe, refractory NLRC4-MAS who showed sustained response to treatment with experimental IL-18 inhibition.
摘要
NLRC4 炎性体过度活跃可导致婴儿发病的巨噬细胞活化综合征和肠炎，伴有异常高的血清白细胞介素-18 水平（NLRC4-MAS）。在此，我们报告了一例重症 NLRC4-MAS 危重症婴儿，其对实验性白细胞介素-18 抑制治疗有持续反应。
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