中华内科杂志2023年1月第62卷第1期Chin J Intem Med,January2023,Vl.62.No.1·23·标准与讨论·风湿性疾病相关噬血细胞综合征诊疗规范王迁·王旖旎2王墙3张缪佳3孙红胜4刘重阳5王昭2赵岩·代表中华医学会风湿病学分会·中国医学科学院北京协和医学院北京协和医院凤湿免疫科国家皮肤与免疫疾病临床医学研究中心疑难重症及罕见病国家重点实验室风湿免疫病学教育部重点实验室，北京100730：2首都医科大学附属北京友谊医院血液内科，北京100050：3南京医科大学第一附属医院风湿免疫科，南京210029：4山东第一医科大学附属省立医院风湿免疫科，济南250021；5重庆医科大学附属第三医院风湿免疫科，重庆401120道信作者：张缪佳，Email:miaojia_zhang(@l63.com;赵岩，Email:zhaoyan_.pumch2002@aliyun.com【摘要】噬血细胞综合征(HPS)又称噬血细胞性淋巴组织细胞增多症(HH),其中继发于风湿性疾病的HLH亦称为巨噬细胞活化综合征(MAS)。HH是细胞毒T细胞和单核-巨噬细胞系统过度激活，临床上以持续发热、肝脾肿大、全血细胞减少及骨髓、肝、脾、淋巴结组织出现噬血现象为特征的综合征。MAS是风湿性疾病的高危并发症，需要多学科密切合作。为提高临床医师对MAS的认识，规范MAS的临床诊治实践，中华医学会风湿病学分会组织国内相关专家制定了本规范，旨在提高MAS的诊治水平，改善患者预后。【关键词】噬血细胞综合征；巨噬细胞活化综合征；淋巴组织细胞增多症，嗜血细胞性；自身免疫病；自身炎症性疾病；诊断；治疗；预后1915Recommendations for the diagnosis and treatment of rheumatic diseases-relatedhemophagocytic syndrome in ChinaWang Qian',Wang Yinr,Wang Qiang',Zhang Miaojia',Sun Hongsheng',Liu Chongyang',Wang Zhao',Zhao Yan',on behalf of Chinese Rheumatology AssociationDepartment of Rheumatology and Clinical Immunology,Peking Union Medical College Hospital,PekingUnion Medical College,Chinese Academy of Medical Sciences,National Clinical Research Center forDermatologic and Immunologic Diseases,State Key Laboratory of Complex Sever and Rare Diseases,Key Laboratory of Rheumatology and Clinical Immunology,Ministry of Education,Beijing 100730,China;*Department of Hematology,Beijing Friendship Hospital,Capital Medical University,Beijing100050,China;Department of Rheumatology and Immunology,the First Affiliated Hospital of NanjingMedical University,Nanjing 210029,China;*Department of Rheumatology and Immunology,ShandongProvincial Hospital Affiliated to Shandong First Medical University,Jinan 250021,China;5 Departmentof Rheumatology and Clinical Immunology,the Third Affiliated Hospital of Chongqing MedicalUniversity,Chongqing 401120,ChinaCorresponding author:Zhang Miaojia,Email:miaojia_zhang@163.com;Zhao Yan,Emailzhaoyan_pumch2002@aliyun.comAbstract Hemophagocytic syndrome (HPS),which is currently named ashemophagocytic lymphohistiocytosis (HLH),is a hyperinflammatory syndrome characterized bypersistent fever,hepatosplenomegaly,pancytopenia and hemophagocytosis found in bone marrow,■D01:10.3760/cma.i.cnl12138-20221010-00749收稿日期2022-10-10本文编辑胡朝晖引用本文：王迁，王旖旎，王嫱，等.风湿性疾病相关噬血细胞综合征诊疗规范[.中华内科杂志，2023，62(1:23-30.D0:10.3760/cma.j.cn112138-20221010-00749
