neonatal lupus erythematosus

  • 文章类型: Journal Article
    这项研究,通过搜索“母体狼疮”等关键词进行“新生儿狼疮”,以及PubMed和Scopus等数据库中的“先天性心脏传导阻滞”,提供了对胎儿和新生儿狼疮的详细叙述回顾。自身抗体如抗Ro/SSA和抗La/SSB可能穿过胎盘并引起新生儿并发症,如先天性心脏传导阻滞(CHB)。管理选择涉及羟氯喹,能够抵消一些不良事件,尽管由于该药物对QTc间期的影响,因此需要谨慎使用。新生儿CHB的高级起搏策略,尤其是在严重的形式,如积水,也进行了评估。这篇综述强调风湿病学家需要跨学科护理,产科医生,和儿科医生,以便在狼疮妊娠中实现最佳的孕产妇和新生儿健康。这种多学科方法旨在改善疾病的结果和管理,减轻母亲和婴儿的负担。
    This study, conducted by searching keywords such as \"maternal lupus\", \"neonatal lupus\", and \"congenital heart block\" in databases including PubMed and Scopus, provides a detailed narrative review on fetal and neonatal lupus. Autoantibodies like anti-Ro/SSA and anti-La/SSB may cross the placenta and cause complications in neonates, such as congenital heart block (CHB). Management options involve hydroxychloroquine, which is able to counteract some of the adverse events, although the drug needs to be used carefully because of its impact on the QTc interval. Advanced pacing strategies for neonates with CHB, especially in severe forms like hydrops, are also assessed. This review emphasizes the need for interdisciplinary care by rheumatologists, obstetricians, and pediatricians in order to achieve the best maternal and neonatal health in lupus pregnancies. This multidisciplinary approach seeks to improve the outcomes and management of the disease, decreasing the burden on mothers and their infants.
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  • 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
    系统性红斑狼疮(SLE),一种主要影响女性的常见自身免疫性疾病,与怀孕期间的各种并发症有关。抗Ro/SSA抗体从受SLE影响的母亲转移到其后代可导致新生儿狼疮和心脏问题。这项研究调查了母体SLE与小儿心血管疾病风险之间的关系。
    该研究利用了韩国的国民健康保险服务(NHIS)数据库,涵盖了2007年至2017年之间出生的3,505,737名儿童,并追踪到2020年。使用世界卫生组织的国际疾病分类第十次修订版(ICD-10)代码确定产妇SLE病例,并与分娩记录相关联。心脏病分为先天性心脏病(CHD),心律失常,和获得性心脏病。将1:4比率匹配的倾向评分应用于设定对照组。
    在3,505,737名儿童中,0.7%(n=23,330)的母亲患有SLE。产妇SLE组早产发生率明显较高(5.9%vs.3.0%)。与对照组相比,SLE母亲所生的孩子患总体CHD的风险显着升高(5.5%,调整后的赔率比[AOR]1.21;95%置信区间[CI]1.14-1.29),包括房间隔缺损(1.18;1.09-1.28)和动脉导管未闭(1.15;1.03-1.30)。此外,在心律失常(完全性房室传导阻滞7.20;2.41-21.49)和获得性心脏病中观察到明显更高的风险,包括心肌病(1.40;1.17-1.68)和粘膜皮肤淋巴结综合征(MCLS)(1.27;1.15-1.43)。
    母体SLE与后代的先天性和获得性心脏病有关,包括结构性的,心律失常,和MCLS。这项研究强调,由于涉及母体自身抗体的多因素影响,这些儿童需要从产前阶段到青春期前进行持续的心血管监测。遗传易感性,和环境因素。
    UNASSIGNED: Systemic lupus erythematosus (SLE), a common autoimmune disease predominantly affecting women, has been linked to various complications during pregnancy. The transfer of anti-Ro/SSA antibodies from SLE-affected mothers to their offspring can lead to neonatal lupus and cardiac issues. This study investigated the association between maternal SLE and the risk of pediatric cardiovascular disorders.
    UNASSIGNED: The study utilized South Korea\'s National Health Insurance Service (NHIS) database, covering 3,505,737 children born between 2007 and 2017 and tracked until 2020. Maternal SLE cases were identified using the World Health Organization\'s International Classification of Diseases Tenth revision (ICD-10) codes and linked with delivery records. Cardiologic disorders were categorized into congenital heart disease (CHD), arrhythmic disorders, and acquired heart disease. Propensity score matching with 1:4 ratios was applied to the set control group.
    UNASSIGNED: Among 3,505,737 children, 0.7% (n = 23,330) were born to mothers with SLE. The incidence of preterm birth was significantly higher in the maternal SLE group (5.9% vs. 3.0%). Compared with the control group, children born to mothers with SLE exhibited a significantly elevated risk of overall CHDs (5.5%, adjusted odds ratio [aOR] 1.21; 95% confidence interval [CI] 1.14-1.29), including atrial septal defect (1.18; 1.09-1.28) and patent ductus arteriosus (1.15; 1.03-1.30). In addition, a notably higher risk was observed in arrhythmic disorders (complete atrioventricular block 7.20; 2.41-21.49) and acquired cardiac disorders, including cardiomyopathy (1.40; 1.17-1.68) and mucocutaneous lymph node syndrome (MCLS) (1.27; 1.15-1.43).
    UNASSIGNED: Maternal SLE is associated with congenital and acquired cardiac disorders in offspring, including structural, arrhythmic, and MCLS. This study highlights the need for continuous cardiovascular monitoring from the prenatal stage to preadolescence in these children due to multifactorial influences involving maternal autoantibodies, genetic predisposition, and environmental factors.
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  • 文章类型: Case Reports
    暂无摘要。
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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: Case Reports
    新生儿红斑狼疮(NLE)是一种自身免疫性疾病,由自身抗体在怀孕期间从母亲到孩子的被动转移引起。NLE的罕见并发症是噬血细胞性淋巴组织细胞增生症(HLH),一种可能危及生命的高炎症状态,通常与其他风湿病相关。在这里,我们描述了NLE相关HLH的致命病例。
    Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by the passive transfer of autoantibodies from mother to child during pregnancy. A rare complication of NLE is hemophagocytic lymphohistiocytosis (HLH), a potentially life-threatening hyperinflammatory state more commonly associated with other rheumatologic disorders. Herein, we describe a fatal case of NLE-associated HLH.
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  • 文章类型: Case Reports
    新生儿红斑狼疮(NLE)是一种罕见的疾病,在美国约有20,000名活产婴儿。NLE的常见表现包括皮肤爆发和心脏受累。NLE的典型皮疹在临床和组织病理学上最类似于亚急性皮肤红斑狼疮的皮疹。我们介绍了一名3个月大的男性与NLE相关的反应性肉芽肿性皮炎(RGD)病例,其中最初的组织病理学和免疫组织化学与血液恶性肿瘤有关。RGD是一个统一的术语,用于描述皮肤肉芽肿爆发,发生在各种刺激的反应,包括自身免疫性结缔组织疾病。我们的病例证明了NLE患者可能存在的组织病理学发现范围。
    Neonatal lupus erythematosus (NLE) is an uncommon disorder affecting approximately one out of 20 000 live births in the United States. Common manifestations of NLE include cutaneous eruptions and cardiac involvement. The typical rash of NLE most closely resembles the rash of subacute cutaneous lupus erythematosus both clinically and histopathologically. We present a case of reactive granulomatous dermatitis (RGD) associated with NLE in a 3-month-old male in whom the initial histopathology and immunohistochemistry were concerning for hematologic malignancy. RGD is a unifying term used to describe cutaneous granulomatous eruptions that occur in response to a variety of stimuli, including autoimmune connective tissue diseases. Our case demonstrates the range of histopathological findings that may be present in the setting of NLE.
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  • 文章类型: Case Reports
    我们报告了三个不相关的人,每个人在妊娠期间暴露于母体自身抗体,并在加利福尼亚州新生儿X连锁肾上腺脑白质营养不良(ALD)筛查(NBS)阳性后,在新生儿期间发现超长链脂肪酸(VLCFAs)升高。两名先证者具有新生儿红斑狼疮(NLE)的临床和实验室特征;第三位具有提示NLE的特征以及干燥综合征和类风湿性关节炎的已知母体病史。在所有三个个体中,随后对原发性和继发性过氧化物酶体疾病的生化和分子评估在15月龄时VLCFA恢复正常后无法诊断.这些病例增加了通过升高的C26:0-溶血磷脂酰胆碱筛查ALD阳性的新生儿的扩大的鉴别诊断。尽管胎盘母体抗Ro抗体如何损伤胎儿组织的病理生理学尚不清楚,我们假设VLCFA升高反映了全身性炎症反应和继发性过氧化物酶体功能障碍,一旦母体自身抗体在出生后减弱,这种情况就会得到改善.对这种现象的额外评估是必要的,以更好地理解复杂的生化,临床,和自身免疫之间可能的治疗重叠,炎症,过氧化物酶体功能障碍,和人类疾病。
    We report three unrelated individuals, each exposed to maternal autoantibodies during gestation and found to have elevated very long-chain fatty acids (VLCFAs) in the newborn period after screening positive by California newborn screening (NBS) for X-linked adrenoleukodystrophy (ALD). Two probands presented with clinical and laboratory features of neonatal lupus erythematosus (NLE); the third had features suggestive of NLE and a known maternal history of Sjogren\'s syndrome and rheumatoid arthritis. In all three individuals, subsequent biochemical and molecular evaluation for primary and secondary peroxisomal disorders was nondiagnostic with normalization of VLCFAs by 15 months of age. These cases add to the expanding differential diagnosis to consider in newborns who screen positive for ALD via elevated C26:0-lysophosphatidylcholine. Though the pathophysiology of how transplacental maternal anti-Ro antibodies damage fetal tissue is not well-understood, we postulate that the VLCFA elevations reflect a systemic inflammatory response and secondary peroxisomal dysfunction that improves once maternal autoantibodies wane after birth. Additional evaluation of this phenomenon is warranted to better understand the intricate biochemical, clinical, and possible therapeutic overlap between autoimmunity, inflammation, peroxisomal dysfunction, and human disease.
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  • 文章类型: Journal Article
    新生儿红斑狼疮(NLE)是一种先天性自身免疫性疾病,其中针对自身抗原的免疫球蛋白G(IgG)经胎盘通过会引起胎儿或新生儿的临床症状。Anti-Ro/SS-A,抗La/SS-B,在较小程度上,抗U1RNP自身抗体(aAbs)与NLE的相关性最强。然而,尽管携带了这些aAbs,但约50%的受影响母亲无症状。该疾病的临床表现非常多样化。心脏表现是最严重的,包括先天性心脏传导阻滞(CHB),死亡率约为18%。羟氯喹(HCQ)的预防性治疗可将随后怀孕的CHB复发率降低约50%。
    Neonatal lupus erythematosus (NLE) is a congenital autoimmune condition in which the transplacental passage of immunoglobulin G (IgG) directed against auto-antigens causes clinical symptoms in the foetus or neonate. Anti-Ro/SS-A, anti-La/SS-B, and to a lesser extent, anti-U1RNP autoantibodies (aAbs) have the strongest association with NLE. However, ~ 50% of affected mothers are asymptomatic despite carrying those aAbs. The clinical picture of the disease is very diverse. Cardiac manifestations are the most severe, including congenital heart block (CHB), with a mortality rate of ~18%. Preventative therapy with hydroxychloroquine (HCQ) reduces the recurrence rate of CHB in subsequent pregnancies by ~50%.
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