Lee L A, Reichlin M, Ruyle S Z, Weston W L
Department of Dermatology, University of Oklahoma Medical School, Oklahoma City.
Lupus. 1993 Oct;2(5):333-8. doi: 10.1177/096120339300200511.
Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by complete congenital heart block and/or transient skin lesions of subacute cutaneous lupus erythematosus. We report that in approximately 10% of cases of NLE with heart block or skin disease, liver disease also occurs (4 of 35 cases in our series). Cholestasis was the major feature in our cases. Although the cholestasis may be severe, the disease process appears to be transient and surviving babies have been healthy on follow-up. In one liver examined for antibody deposition, IgG antibody deposits, presumably of maternal origin, were present. Three maternal sera were examined for autoantibodies, including liver-specific autoantibodies. No liver-specific autoantibodies were found. Rather, the maternal autoantibodies too were the ubiquitous Ro/SSA-associated autoantigens. The autoantibodies bound the 60 kDa SSA/Ro ribonuclear protein (three of three sera), the 52 kDa SSA/Ro protein (two of three sera) and the SSB/La ribonuclear protein (two of three sera).
新生儿红斑狼疮（NLE）是一种自身免疫性疾病，其特征为完全性先天性心脏传导阻滞和/或亚急性皮肤型红斑狼疮的短暂性皮肤损害。我们报告，在约10%患有心脏传导阻滞或皮肤病的NLE病例中，也会出现肝脏疾病（我们系列研究中的35例中有4例）。胆汁淤积是我们病例中的主要特征。尽管胆汁淤积可能很严重，但病程似乎是短暂的，存活下来的婴儿在随访中一直健康。在一个检查抗体沉积的肝脏中，存在推测为母体来源的IgG抗体沉积物。检测了三份母体血清中的自身抗体，包括肝脏特异性自身抗体。未发现肝脏特异性自身抗体。相反，母体自身抗体也是普遍存在的与Ro/SSA相关的自身抗原。这些自身抗体与60 kDa SSA/Ro核糖核蛋白（三份血清中的三份）、52 kDa SSA/Ro蛋白（三份血清中的两份）和SSB/La核糖核蛋白（三份血清中的两份）结合。
