Tseng C E, Buyon J P
Department of Medicine, New York University School of Medicine, New York, USA.
Rheum Dis Clin North Am. 1997 Feb;23(1):31-54. doi: 10.1016/s0889-857x(05)70313-6.
Congenital heart block is considered to be a model of passively acquired autoimmunity, whereby immune abnormalities in the mother lead to the production of autoantibodies that cross the placenta and presumably injure the otherwise normally developing fetus. The major targets of the maternal immune response are the SSA/Ro and SSB/La ribonucleoproteins. Other neonatal abnormalities affecting the skin, liver, and blood elements have also been reported to be associated with anti-SSA/Ro-SSB/La antibodies in the maternal and fetal circulation and are now grouped along with congenital heart block under the heading of the neonatal lupus syndromes. This review covers the histopathology, SSA/Ro-SSB/La antigen-antibody systems, immunogenetics, clinical manifestations, and diagnosis and management strategies of these syndromes.
先天性心脏传导阻滞被认为是被动获得性自身免疫的一个模型，即母亲的免疫异常导致自身抗体的产生，这些抗体穿过胎盘并可能损害原本正常发育的胎儿。母体免疫反应的主要靶点是SSA/Ro和SSB/La核糖核蛋白。据报道，其他影响皮肤、肝脏和血液成分的新生儿异常也与母体和胎儿循环中的抗SSA/Ro - SSB/La抗体有关，现在它们与先天性心脏传导阻滞一起被归类为新生儿狼疮综合征。这篇综述涵盖了这些综合征的组织病理学、SSA/Ro - SSB/La抗原 - 抗体系统、免疫遗传学、临床表现以及诊断和管理策略。
