neonatal lupus erythematosus

  • Neonatal lupus erythematosus (NLE) is caused by the transmission of maternal anti-Ro/SSA antibodies, anti-La/SSB antibodies, and other autoantibodies to the fetus through the placenta. Usually, with the disappearance of autoantibodies in the children\'s body, abnormal changes in the mucocutaneous, blood system, and digestive system can spontaneously subside, but the damage to various systems caused by autoantibodies may persist for a long time. This article provides a comprehensive review of the manifestations and prognosis of NLE in various systems, including mucocutaneous, blood system, circulatory system, nervous system, digestive system, respiratory system, aiming to provide reference for clinical work.
    新生儿红斑狼疮(neonatal lupus erythematosus, NLE)是由母体的抗-Ro/SSA抗体、抗-La/SSB抗体等自身抗体通过胎盘传给胎儿所致,通常随着患儿体内自身抗体的消失,皮肤黏膜、血液及消化系统等的异常改变可自行消退,但由于自身抗体所导致的各系统损害可能长期存留。该文综述了NLE在皮肤黏膜、血液系统、循环系统、神经系统、消化系统、呼吸系统等方面的表现及预后的研究进展,以期为临床工作提供参考。.
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  • 文章类型: Journal Article
    背景:新生儿红斑狼疮(NLE)是一种罕见的自身免疫性疾病,需要区分湿疹,先天性梅毒,和新生儿的体癣。反射共聚焦显微镜(RCM)可能是一种有用的非侵入性诊断工具,已用于评估几种炎症性皮肤状况。本研究的目的是描述NLE的RCM特征。
    方法:将9例NLE患者纳入研究,所有患者均采用RCM进行临床评估.我们还评估了9例湿疹患者作为对照的RCM图像。
    结果:RCM可以观察到NLE的一些主要关键诊断特征,扩大的蜂窝图案(7/9,78%),存在圆形至卵圆形囊肿样结构(6/9,67%),正常的环状结构在真皮-表皮交界处完全或部分消失(9/9,100%),中等折射率杂乱无章的粗纤维材料(8/9,89%),大量高折射率圆形细胞(9/9,100%)在真皮。
    结论:RCM可以可视化NLE的主要关键诊断特征,并作为NLE的补充诊断工具。
    BACKGROUND: Neonatal lupus erythematosus (NLE) is a rare autoimmune disease, which needs to be distinguished from eczema, congenital syphilis, and tinea corporis in newborns. Reflectance confocal microscopy (RCM) could be a helpful noninvasive diagnostic tool, which has been used to evaluate several inflammatory skin conditions. The aim of this study was to describe the RCM characteristics of NLE.
    METHODS: Eleven NLE patients were included in the study, and all patients were evaluated clinically with RCM. We also evaluated RCM images from 11 eczema patients as controls.
    RESULTS: Some major key diagnostic features of NLE can be observed by RCM: an enlarged honeycomb pattern (9/11, 81.8%), round-to-oval cyst-like structures were present (6/11, 54.5%), the normal ring-like structures were totally or partially obliterated (11/11, 100%) at the level of the dermo-epidermal junction, medium refractivity collagen fibers that were disorganized (10/11, 90.9%), numerous high refractivity round cells (11/11, 100%) in the dermis.
    CONCLUSIONS: RCM allows the visualization of major key diagnostic features of NLE and serves as a complementary diagnostic tool for NLE.
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  • 文章类型: Journal Article
    本研究旨在分析足月新生儿红斑狼疮(NLE)高危人群的临床特点及危险因素。母亲抗SSA阳性的NLE婴儿的高危人群,纳入了妊娠期抗SSB或抗U1RNP抗体.他们出生在2013年2月至2020年2月之间,胎龄不低于37周。我们分析了他们从出生到出生后24个月的临床数据。共纳入105例NLE高危患者。其中,30例患者被诊断为NLE(NLE组),75例患者没有(非NLE组)。NLE组受影响的系统包括真皮(13.3%),肝(76.0%),和血液系统(43.3%)。肝脏受累,贫血和血小板减少症直到60天才出现,出生后41天和22天,分别,在某些情况下。全身受累可在出生后3至12个月内治愈。特异性自身抗体的清除时间为出生后12个月。两组婴儿及其母亲的临床特征无显著差异,无论是阳性率还是特异性自身抗体的清除时间。
    结论:经过标准化的产前保健,皮肤的风险仍然很高,肝,或NLE高危人群的血液系统受累。没有具体的指标来预测婴儿是否会发展为NLE。所有这些患者都需要在出生后一年内密切随访。
    背景:•新生儿红斑狼疮(NLE)可影响心脏,真皮,肝,和婴儿的血液系统。
    背景:•在我们中心采用良好的多部门合作进行标准化的产前保健之后,在这项研究中,没有新生儿出现心脏传导阻滞.然而,真皮,肝,NLE的血液系统受累在随访期间仍可逐渐出现(在某些情况下长达出生后60天),其中一些情况很严重,需要及时积极的干预。没有发现单一因素可以预测母亲抗SSA阳性的NLE高危后代是否患有NLE,SSB和/或RNP将发展NLE。
    This study aims to analyze the clinical characteristics and risk factors of high-risk groups of neonatal lupus erythematosus (NLE) in term infants. High-risk groups of NLE infants whose mothers were positive for anti-SSA, anti-SSB or anti-U1RNP antibodies during pregnancy were enrolled. They were born between February 2013 and February 2020, with a gestational age not less than 37 weeks. We analyzed their clinical data from birth to 24 months after birth. A total of 105 patients in the NLE high-risk group were included. Among them, 30 patients were diagnosed with NLE (NLE group), and 75 patients were not (non-NLE group). The affected systems of the NLE group included the dermal (13.3%), hepatic (76.0%), and hematological systems (43.3%). Hepatic involvement, anemia and thrombocytopenia did not emerge until 60 days, 41 days and 22 days after birth, respectively, in some cases. Systemic involvement could be cured within 3 to 12 months after birth. The clearance time of specific autoantibodies was 12 months after birth. There was no significant difference in the clinical characteristics of babies and their mothers between the two groups, neither in the positive rate nor in the clearance time of specific autoantibodies.
    CONCLUSIONS: After standardized prenatal health care, there is still a high risk of dermal, hepatic, or hematological system involvement for high-risk groups of NLE. There are no specific indicators for the prediction of whether babies will develop NLE. All of these patients need to be followed up closely within one year after birth.
    BACKGROUND: • Neonatal lupus erythematosus (NLEs) can affect the cardiac, dermal, hepatic, and hematological systems of infants.
    BACKGROUND: • After standardized prenatal health care employing good multidepartment cooperation in our center, no neonates had cardiac block in this study. However, dermal, hepatic, and hematological system involvement of NLE can still gradually appear (as long as 60 days after birth in some cases) during follow-up, and some of these conditions are serious and require timely and active intervention. No single factor has been found to predict whether offspring at high-risk of NLE whose mothers are positive for anti-SSA, SSB and/or RNP will develop NLE.
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  • 文章类型: Journal Article
    UNASSIGNED: To explore the clinical features, autoantibodies, and outcome of neonatal lupus erythematosus (NLE). Methods: We retrospectively reviewed all NLE cases from January 2012 to May 2019 that occurred in our department. Results: Cutaneous, cardiac, hematologic, and hepatobiliary manifestations were found in 36.7%, 56.7%, 56.7%, and 30.0% of cases, respectively. The presence of anti-SSA antibodies was correlated with cardiac presentation (p = .026) and the presence of anti-SSB antibodies was associated with cutaneous lesions (p = .015). During the follow-up, one patient with a third-degree atrioventricular block died, and a third-degree AV block persists in a child without a pacemaker at 4-years of age. No other manifestations of NLE were observed after the age of 12 months. Conclusions: Anti-SSA and anti-SSB are associated with cardiac and cutaneous manifestations in NLE. Most children with NLE have excellent outcomes with symptom resolution by one year. Complete congenital heart block may persist.
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  • 文章类型: Journal Article
    The aims of our study are to observe the pregnancy outcome of anti-Sjögren\'s syndrome-related antigen A (SSA)/Ro-positive women and to predict the risk factors for adverse pregnancy outcome and neonatal lupus erythematosus (NLE). Clinical data of 126 anti-SSA/Ro-positive patients with 140 pregnancies were evaluated retrospectively, and the newborns were followed up as a cohort in 3 months. χ (2) test or logistic regression was used to predict the risk factors of lupus flares during pregnancy, fetal loss, and NLE. Twenty-six out of 93 pregnancies with systemic lupus erythematosus (SLE) experienced flares during pregnancy. Active disease prior to conception was the independent risk factor for flares [P = 0.002, odds ratio (OR) = 10.41 (95 % confidence interval (CI) = 2.34∼46.26)]. Continuous use of steroids and hydroxychloroquine (HCQ) might help decrease the risk (P = 0.041 and 0.015, respectively). Eleven out of 140 pregnancies ended with fetal loss, and 9 out of 113 live births were diagnosed with NLE. The presence of anti-phospholipid syndrome (APS) was associated with fetal loss (P = 0.018, OR = 6.41 (95 % CI = 1.57-26.14)). The presence of anti-Sjögren\'s syndrome-related antigen B (SSB)/La antibodies tended to increase the risk of giving birth to an infant with NLE (P = 0.140); on the other hand, duration of disease, history of renal involvement, and active SLE during pregnancy did not contribute to the incidence of NLE (P = 0.649, 0.685, and 1.000, respectively). Active disease without regular follow-up before conception significantly increased the risk of lupus flares during pregnancy. The continuous use of low-dose steroids and hydroxychloroquine might help maintain lower SLE activity. Concurrent APS instead of high titer of anti-SSA/Ro might raise the risk of fetal loss in anti-SSA/Ro-positive patients.
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  • 文章类型: Journal Article
    OBJECTIVE: To analyze the clinical features, outcomes and prognosis of neonatal lupus erythematosus (NLE) in China.
    METHODS: We reviewed 12 NLE cases at the Peking Union Medical College Hospital and compared the data with 111 cases reported in China between 1990 and 2014. The Chinese medical journal search engines used in this study were Wanfang.data and Science China.
    RESULTS: No gender dominance in NLE incidence was found. Cutaneous lesions were present in more than 96% of patients, while cardiac, hematological and hepatobiliary manifestations were seen in 12.61%, 45.53% and 17.89% of cases, respectively. Congenital heart block (CHB) tended to be more persistent, with two cases showing CHB for 1 year and three cases persisting for 7-10 years. In this study more than 90% of mothers were anti-Sjögren\'s syndrome A positive, and 65.04% were asymptomatic prior to the pregnancy.
    CONCLUSIONS: These results indicate that clinicians, especially dermatologists, in China should improve their recognition of this disease to avoid misdiagnosis, and more attention should be paid to the follow-up of NLE patients and their asymptomatic mothers.
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