Rheumatology Unit, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
Scand J Immunol. 2010 Sep;72(3):185-8. doi: 10.1111/j.1365-3083.2010.02442.x.
Congenital heart block is the most severe manifestation of neonatal lupus syndrome. It is a passively acquired disease where transplacental passage of maternal autoantibodies is associated with irreversible damage of the foetal cardiac conduction system. It is well established that the condition, in the absence of structural abnormalities, is strongly associated with maternal autoantibodies to the Ro/La antigens. More specifically the disease has been closely linked to antibodies to the Ro52 component of the antigen complex. Congenital heart block constitutes a unique model where specific autoantibodies target and mediate organ-specific disease. A wide panel of maternal antibodies has been discussed in literature in association with the disease and are described in this review.
先天性心脏传导阻滞是新生儿狼疮综合征最严重的表现。它是一种被动获得的疾病，母体自身抗体通过胎盘传递与胎儿心脏传导系统的不可逆损伤有关。已经确定，在没有结构异常的情况下，这种情况与母体自身抗体对 Ro/La 抗原强烈相关。更具体地说，该疾病与抗原复合物的 Ro52 成分的抗体密切相关。先天性心脏传导阻滞构成了一个独特的模型，其中特定的自身抗体针对并介导器官特异性疾病。本文综述了文献中讨论的与该疾病相关的多种母体抗体。
