Lupus-like autoimmunity and increased interferon response in patients with STAT3-deficient hyper-IgE syndrome

Rishi R Goel,Shuichiro Nakabo,Brian L P Dizon,Amanda Urban,Meryl Waldman, Lillian Howard,Dirk Darnell,Munir Buhaya,Carmelo Carmona-Rivera,Sarfaraz Hasni,Mariana J Kaplan,Alexandra F Freeman,Sarthak Gupta

Journal of Allergy and Clinical Immunology（2021）

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摘要

We report on patients with STAT3 loss-of-function (LOF) mutations who developed lupus-like autoimmunity. Immune dysregulation seen in STAT3 LOF patients suggests a susceptibility to systemic autoimmunity with important implications in monitoring and management of these patients.

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关键词

NETs,STAT3,autoimmunity,hyper-IgE,interferon,lupus,neutrophils

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