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中国新生儿红斑狼疮临床诊疗专家共识(2025)OA

Expert consensus on clinical diagnosis and management of neonatal lupus erythematosus in China(2025)

中文摘要英文摘要

新生儿红斑狼疮(neonatal lupus erythematosus,NLE)是一种妊娠期间由母体的自身抗体经胎盘传递给胎儿导致的获得性自身免疫性疾病.当母亲或患儿存在抗干燥综合征A抗体(SSA/Ro抗体)、B抗体(SSB/La抗体)等自身抗体,或母亲患有自身免疫性疾病,且胎儿或新生儿出现心脏、皮肤、肝脏或血液系统症状,但无法用其他原因解释时,则可诊断为NLE.NLE 的心外症状轻微且具有自限性,通常不需要治疗.而心脏病变预后较差,尤其是先天性心脏传导阻滞(congenital heart block,CHB),病死率为15%~30%.因此预防CHB成为NLE临床诊疗的核心任务,这既包括在胎儿期进行及时、有效的心脏监测和药物干预,也包括CHB新生儿的药物治疗和起搏器植入.为了提高CHB患儿的生存率和改善预后,基于国内外证据,中国医药教育协会新生儿专业委员会发起制定本共识,更新推荐了抗SSA阳性孕妇孕12周前启动羟氯喹400 mg·d-1预防,细化低出生体重儿心外膜起搏指征,提出抗p200抗体检测策略.

Neonatal lupus erythematosus(NLE)is an acquired autoimmune disorder caused by the transplacental transfer of maternal autoantibodies to the fetus during pregnancy.NLE is diagnosed when the mother or infant tests positive for autoantibodies such as anti-Sjögren's syndrome A(SSA/Ro)or B(SSB/La)antibodies,or the mother has autoimmune diseases;and the fetus or neonate presents with cardiac,cutaneous,hepatic,or hematologic manifestations unexplained by other etiologies.Extracardiac manifestations of NLE are typically mild and self-limiting,often requiring no treatment.In contrast,cardiac involvement-particularly congenital heart block(CHB)carries a poor prognosis,with a mortality rate from 15%to 30%.Thus,CHB prevention has become the cornerstone of NLE management,encompassing both fetal cardiac surveillance and pharmacologic interventions during pregnancy,as well as postnatal therapies(e.g.,medication treatment and pacemaker implantation)for affected neonates.To improve survival and outcomes in CHB,the Neonatal Committee of the China Medicine Education Association developed this consensus,incorporating international evidence.Key recommendations include Hydroxychloroquine(400 mg·d-1)initiation before 12 gestational weeks for anti-SSA/Ro-positive pregnant women,updated criteria for epicardial pacing in low-birth-weight infants and anti-p200 antibody testing strategies.

中国医药教育协会新生儿专业委员会;崔红

首都医科大学附属北京友谊医院

红斑狼疮自身免疫性疾病自身抗体先天性心脏传导阻滞新生儿

Lupus erythematosusAutoimmune diseasesAutoantibodiesCongenital heart blockNewborn

《中国循证儿科杂志》 2026 (1)

1-7,7

首都医科大学2024年研究项目:2024JYY147

10.3969/j.issn.1673-5501.2026.01.001

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