Costedoat-Chalumeau N, Amoura Z, Villain E, Cohen L, Fermont L, Le Thi Huong D, Vauthier D, Georgin-Lavialle S, Wechsler B, Dommergues M, Piette J-C
Service de Médecine Interne, CHU Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75651 Paris Cedex 13.
J Gynecol Obstet Biol Reprod (Paris). 2006 Apr;35(2):146-56. doi: 10.1016/s0368-2315(06)76388-8.
Fetuses and infants of women with anti-SSA/Ro and anti-SSB/La antibodies are at risk of neonatal lupus syndrome, featuring skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. The prevalence of CHB in newborns of anti-SSA/Ro positive women with known connective tissue disease is 1 to 2% and the risk of recurrence ranges from 10 to 17%. CHB is definitive and is associated with significant morbidity (pacemaker must be implanted in 2/3 of cases) and mortality (16 to 19%). Myocardial involvement may either be associated or appear subsequently. Other manifestations are discussed. For anti-SSA/Ro positive pregnant women, echocardiograms should be performed every 2 weeks from 16 to 24 weeks of gestation, and every week in case of past history of CHB. Electrocardiogram should be performed in the first days of life for all children to detect incomplete CHB. Therapy for CHB detected in utero is based on fluorinated steroids, especially betamethasone. Its efficiency is variable.
患有抗SSA/Ro和抗SSB/La抗体的女性所孕育的胎儿和婴儿有患新生儿狼疮综合征的风险，其特征为皮肤病变、血液系统和肝脏疾病，以及在无严重心脏畸形情况下的先天性心脏传导阻滞（CHB）。已知患有结缔组织病的抗SSA/Ro阳性女性所生新生儿中CHB的患病率为1%至2%，复发风险为10%至17%。CHB是确定性的，且与显著的发病率（三分之二的病例必须植入起搏器）和死亡率（16%至19%）相关。心肌受累可能与之相关或随后出现。还讨论了其他表现。对于抗SSA/Ro阳性的孕妇，应在妊娠16至24周期间每2周进行一次超声心动图检查，如有CHB病史则每周进行一次。所有儿童在出生后的头几天应进行心电图检查以检测不完全性CHB。宫内检测到CHB的治疗基于氟化类固醇，尤其是倍他米松。其疗效不一。
