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首页> 外文期刊>Clinics in dermatology >Neonatal lupus erythematosus.
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Neonatal lupus erythematosus.

机译：新生儿红斑狼疮。

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Neonatal lupus Erythematosus (NLE) is a disorder characterized by maternal autoantibodies against RNA protein complex, Ro/SSA or SSB/La. These maternal IgG antibodies cross the placenta and potentially lead to fetal tissue damage and the clinical manifestations NLE. NLE is uncommon, affects females more than males, has no race predilection, and involves multiple organs. It has cutaneous manifestations similar to subacute cutaneous lupus erythematosus (SCLE). In addition to skin findings, patients with NLE have a significant risk of congenital heart block (CHB), a potentially fatal complication. Less frequently, hematologic and hepatic abnormalities occur. Approximately half of the reported cases have skin disease and half have CHB. Approximately 10% have both CHB and skin findings. The cutaneous, hematologic, and hepatic abnormalities are transient, clearing by 6 months of age. However, CHB is permanent and requires a pacemaker in many cases. The disorder results from the passive transfer of maternal autoantibodies, anti-RoSSA and anti-La/SSB. Sontheimer and McCauliffe reviewed the pathogenic role of anti-Ro antibody in NLE lesions and summarize evidence supporting its pathogenic role. Additional evidence suggests the possibility that Ro-antigen-specific T-cells may be present in SCLE patients and have the capacity to cause direct injury to the skin. McCuistion and Schoch first suggested this hypothesis in 1954 when they described an infant with LE skin findings born to a mother with systemic lupus erythematosus (SLE). Since this initial observation, a number of researchers have documented the role of maternal autoantibodies in the pathogenesis of this disorder. The true incidence of NLE is not known; however, it is known that NLE accounts for approximately 80% of all cases of CHB, and the incidence of CHB is 1 per 20,000 live births. Therefore, it can be assumed that the incidence of NLE is at least 1 per 12,500 live births.
机译：新生儿红斑狼疮（NLE）是一种特征为母体针对RNA蛋白复合物，Ro / SSA或SSB / La的自身抗体的疾病。这些母体IgG抗体穿过胎盘，并可能导致胎儿组织损伤和临床表现NLE。 NLE不常见，对女性的影响大于男性，没有种族偏爱，涉及多个器官。它具有类似于亚急性皮肤性红斑狼疮（SCLE）的皮肤表现。除皮肤病征外，NLE患者还患有先天性心脏传导阻滞（CHB）的重大风险，这是一种潜在的致命并发症。血液和肝异常发生的频率较低。大约一半的报告病例患有皮肤病，另一半患有CHB。大约10％的人同时患有CHB和皮肤。皮肤，血液和肝脏异常是暂时性的，到6个月大时会清除。但是，CHB是永久性的，在许多情况下需要起搏器。该疾病是由母体自身抗体，抗RoSSA和抗La / SSB的被动转移引起的。 Sontheimer和McCauliffe综述了抗Ro抗体在NLE病变中的致病作用，并总结了支持其致病作用的证据。其他证据表明，SCLE患者中可能存在Ro抗原特异性T细胞，并具有对皮肤造成直接伤害的能力。 McCuistion和Schoch于1954年首次提出了这一假设，当时他们描述了一名患有系统性红斑狼疮（SLE）的母亲所生的LE皮肤表现婴儿。自从这一初步观察以来，许多研究人员已证明母体自身抗体在该疾病发病机理中的作用。 NLE的真正发病率尚不清楚;然而，众所周知，在所有慢性乙型肝炎病例中，非酒精性脂肪性肝炎约占80％，而慢性乙型肝炎的发病率为每20,000例活产中有1例。因此，可以假设每12500例活产中NLE的发生率至少为1。

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