新生儿红斑狼疮相关进展研究

Study on the Progress Related to Neonatal Lupus Erythematosus

摘要: 新生儿红斑狼疮(Neonatal lupus erythematosus, NLE)是一种罕见的结缔组织病，可以影响多个器官，包括皮肤、心脏、肝脏、骨髓和中枢神经系统。其发病机制与母体内自身抗体(抗干燥综合征A抗体和抗干燥综合征B抗体)通过胎盘进入胎儿体内引起的多器官、系统免疫损伤有关。NLE的主要临床表现是皮肤损害和先天性心脏传导阻滞(Congenital heart block, CHB)，也可能累及肝脏和血液系统。罕见的情况下，多个系统同时受累。先天性心脏传导阻滞是NLE最严重的表现，病死率高，预后较差。因此，对具有高危因素的母亲，应加强围产期管理，以便早期诊断和干预。为了改善NLE患儿的预后，我们需要进一步深入研究其发病机制和防治策略。本综述对新生儿红斑狼疮在病因、临床表现、治疗和预后等方面的研究进展进行的总结。

Abstract: Neonatal lupus erythematosus (NLE) is a rare connective tissue disease that can affect multiple organs, including skin, heart, liver, bone marrow, and central nervous system. Its pathogenesis is related to the multi-organ and systemic immune damage caused by maternal autoantibodies (anti-Sjögren syndrome A antibodies and anti-Sjögren syndrome B antibodies) entering the fetus through the placenta. The main clinical manifestations of NLE are skin lesions and congenital heart block (CHB), which may also involve the liver and blood system. Rarely, multiple systems are affected simultaneously. Congenital heart block is the most serious manifestation of NLE, with high mortality and poor prognosis. Therefore, perinatal management should be strengthened for mothers with high-risk factors to facilitate early diagnosis and intervention. In order to improve the prognosis of children with NLE, we need to further study its pathogenesis and prevention and treatment strategies. This review summarizes the research progress on the etiology, clinical manifestations, treatment and prognosis of neonatal lupus erythematosus.