自身炎症性疾病与巨噬细胞活化综合征的亲疏  

Autoinflammatory disorders and macrophage activation syndrome:Hand-in-hand or affinity

作　　者：胡坚[1] 李赫[1] 

机构地区：[1]天津市儿童医院免疫暨风湿病学科,天津300134

出　　处：《中国实用儿科杂志》2018年第1期42-45,共4页Chinese Journal of Practical Pediatrics

摘　　要：单基因缺陷自身炎症性疾病(AIDs)实体之间是异质性的,合并巨噬细胞活化综合征(MAS)少见。相似促炎性细胞因子谱研究AIDs-MAS、s JIA-MAS和HLH之间的临床与免疫病理机制可能会有新发现。Monogenic autoinflammatory disorders （AIDs） is a group of heterogeneous diseases, even if macrophage activation syndrome （MAS） and AIDs share as well as heterogeneous pathogenetic scenes, which have the similar clinical features on the occurrence of MAS in the patient with overblown release of proinflammatory cytokines in the natural history. Is the relation between AIDs and MAS？

关 键 词：自身炎症性疾病 巨噬细胞活化综合征 噬血细胞淋巴组织细胞增生症 自身免疫 促炎症细胞因子 

分 类 号：R72[医药卫生—儿科]