Morel N, Georgin-Lavialle S, Levesque K, Guettrot-Imbert G, Le Guern V, Le Bidois J, Bessières B, Brouzes C, Le Mercier D, Villain E, Maltret A, Costedoat-Chalumeau N
Centre de référence maladies auto-immunes et systémiques rares, service de médecine interne pôle médecine, hôpital Cochin, AP-HP, université René-Descartes Paris V, 27, rue du Faubourg-Saint-Jacques, 75679 Paris cedex 14, France.
Service de médecine interne, hôpital Tenon, université Paris VI, 4, rue de la Chine, 75970 Paris cedex 20, France.
Rev Med Interne. 2015 Mar;36(3):159-66. doi: 10.1016/j.revmed.2014.07.013. Epub 2014 Sep 17.
Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.
新生儿狼疮综合征与母体抗SSA/Ro和抗SSB/La抗体经胎盘传递有关。患儿表现出皮肤、血液、肝脏或心脏方面的特征。心脏表现包括先天性心脏传导阻滞（CHB）；心内膜弹力纤维增生症和扩张型心肌病。已知患有结缔组织病的抗Ro/SSA阳性女性新生儿中CHB的患病率在1%至2%之间，复发风险约为19%。皮肤和全身病变是暂时的，而CHB是永久性的，且伴有严重的发病率和18%的死亡率。三分之二的病例必须植入起搏器。心肌炎可能与之相关或随后出现。患有CHB的患儿母亲通常无症状，或表现为干燥综合征或未分化结缔组织病。对于抗Ro/SSA阳性的孕妇，应在妊娠第16至24周期间至少每2周进行一次胎儿超声心动图检查。所有新生儿都应进行心电图检查。宫内检测到的CHB采用氟化皮质类固醇治疗的益处尚不清楚。母体使用羟氯喹可能会降低后续子代CHB复发风险。目前正在进行一项前瞻性研究以证实这些发现。
