背景:新生儿红斑狼疮是由于母体抗Sjögren综合征相关抗原A(抗SSA/Ro)经胎盘通过而在胎儿期间获得的一种自身免疫性疾病,抗干燥综合征相关抗原B(抗SSB/La)或抗U1核糖核蛋白(抗U1-RNP)抗核自身抗体。
背景:临床表现包括皮肤病变,先天性心脏传导阻滞,肝胆受累和血细胞减少。大多数疾病在母体抗体清除后自发消失。心脏症状,然而,不能自我解决,通常需要植入起搏器。诊断基于临床表现和母亲或婴儿血清中典型抗体的存在。
结论:新生儿红斑狼疮可能发生在患有各种系统性结缔组织疾病的抗SSA/Ro或抗SSB/La妇女的儿童中。然而,在一半的案例中,母亲无症状,这可能会延迟诊断,并对儿童的预后产生负面影响。每个孕妇都应考虑检测抗核抗体,因为羟氯喹或静脉注射免疫球蛋白(IVIG)的早期治疗已被证明可有效预防先天性心脏传导阻滞。
BACKGROUND: Neonatal lupus erythematosus is an autoimmune disease acquired during fetal life as a result of transplacental passage of maternal anti-Sjögren\'s-syndrome-related antigen A (anti-SSA/Ro), anti-Sjögren\'s-syndrome-related antigen B (anti-SSB/La) or anti-U1 ribonucleoprotein (anti-U1-RNP) antinuclear autoantibodies.
BACKGROUND: Clinical manifestations include skin lesions, congenital heart block, hepatobiliary involvement and cytopenias. Most of the disorders disappear spontaneously after clearance of maternal antibodies. Cardiac symptoms, however, are not self-resolving and often pacemaker implantation is required. Diagnosis is based on clinical presentation and the presence of typical antibodies in the mother\'s or infant\'s serum.
CONCLUSIONS: Neonatal lupus erythematosus may develop in children born to anti-SSA/Ro or anti-SSB/La women with various systemic connective tissue diseases. However, in half of the cases, the mother is asymptomatic, which may delay the diagnosis and have negative impact on the child\'s prognosis. Testing for antinuclear antibodies should be considered in every pregnant woman since early treatment with hydroxychloroquine or intravenous immunoglobulin (IVIG) has proven to be effective in preventing congenital heart block.