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: 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
    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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  • 文章类型: Journal Article
    OBJECTIVE: To identify the prenatal predisposing factors related to neonatal lupus erythematosus (NLE).
    METHODS: A retrospective case-control study was made of 131 pregnant women with positive anti-Ro or anti-La autoantibodies and known neonatal outcomes between January 2002 and December 2019 at Siriraj Hospital, Bangkok, Thailand. There were 101 unaffected neonates and 30 NLE cases confirmed postnatally. Demographic and clinical data of the mothers and neonates with and without NLE were statistically compared.
    RESULTS: NLE was diagnosed in 30 out of 131 cases. A multivariate analysis identified the following significant factors for NLE: maternal anti-La antibodies (odds ratio (OR), 3.591; p = 0.030); and maternal treatment with either hydroxychloroquine (OR, 0.082; p = 0.001) or prednisolone (OR, 0.136; p = 0.017). Of the significant variables examined in the multivariate analysis models, high levels of maternal anti-La antibodies were found to be the strongest predictor of noncardiac NLE (OR, 4.558; p = 0.032), while a female baby was significantly higher in pregnancies complicated by cardiac NLE (OR, 5.374; p = 0.046). Hydroxychloroquine still provided a protective effect for both cardiac and noncardiac NLE (p = 0.039 and 0.032, respectively).
    CONCLUSIONS: The maternal anti-La antibodies were a beneficial predictor for NLE, especially as their high titers were influentially associated with noncardiac features. A female fetus seemed to present an increased risk for developing a congenital heart block. Nevertheless, the treatment with hydroxychloroquine during the pregnancies demonstrated a potentially protective factor against both cardiac and noncardiac manifestations.
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  • 文章类型: Journal Article
    The purpose of the study was to determine the clinical features of NLE and to compare the neonatal outcomes between newborns born to pregnant women with SLE and healthy pregnant women.
    We conducted a retrospective cohort analysis between 2007 and 2019 in a tertiary referral hospital in Thailand. A total of 118 pregnant women with SLE with 132 neonates compared with 264 randomly selected healthy pregnant women.
    The median (interquartile range) gestational age and birth weight of 132 neonates born to women with SLE were 37 (35-38) weeks and 2687 g (2045-3160 g), respectively. The clinical features of NLE infants were hemolytic anemia (8%), thrombocytopenia (2.7%) and hyperbilirubinemia (5.3%). There was no neonate with a congenital complete heart block or skin lesion. Moreover, logistic regression analysis found that neonates born to women with SLE increased the risk of preterm birth [odd ratio (OR) 8.87, 95% confidence interval (95% CI) 4.32-18.21, p < 0.001], low birth weight (OR 10.35, 95% CI 5.08-21.08, p < 0.001), birth asphyxia (OR 2.91, 95% CI 1.26-6.73, p = 0.011) and NICU admission (OR 4.26, 95% CI 2.44-7.42, p < 0.001). SLE disease activity and corticosteroid and azathioprine usage were associated with preterm delivery in pregnant women with SLE.
    The major clinical features of NLE patients were hematologic and hepatobiliary abnormalities in our study. Pregnancies with SLE dramatically increased the risk of preterm delivery and neonatal complications.
    Neonatal lupus erythematosus (NLE) is the consequence of the transplacental passage of autoantibodies to newborns during pregnancy. The clinical features of NLE infants in our study were hemolytic anemia (8%), thrombocytopenia (2.7%) and hyperbilirubinemia (5.3%). There was no neonate with a congenital complete heart block or skin lesion. We also compared the neonatal outcomes between 118 pregnant women with SLE and 264 randomly selected healthy pregnant women. Our study found that the neonates born to women with SLE increased the risk of preterm birth, low birth weight, birth asphyxia and NICU admission. Moreover, SLE disease activity and corticosteroid and azathioprine usage were associated with preterm delivery in pregnant women with SLE.
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  • 文章类型: Journal Article
    新生儿红斑狼疮(NLE)是一种罕见的疾病,由母亲被动传播的自身抗体引起。NLE是一种以皮肤为特征的多器官系统疾病,心脏,血液学,肝胆,和神经表现。本研究旨在回顾NLE婴幼儿及其母亲的各种症状和临床表现。我们对2009年1月至2020年12月在釜山国立大学儿童医院接受检查和治疗的NLE患者及其母亲的病历进行了回顾性审查。27例NLE患者包括13例男性患者(48.1%)和14例女性患者(51.9%)。最常见的症状是皮疹(40.7%),其次是发烧(25.9%),心律失常(14.8%),脾肿大(11.1%),和宫内发育迟缓(7.4%)。7例发热患者有各种器官系统表现,包括皮肤(100%),血液学(71.4%),肝胆(57.1%),中枢神经系统(CNS;28.6%)表现。发热患者中有两名患有无菌性脑膜炎。皮肤,心脏,血液学,肝胆,中枢神经系统受累占44.4%,18.5%,51.9%,40.7%,和22.2%的患者,分别。系统性红斑狼疮(SLE)是最常见的孕产妇疾病(14/27,51.9%)。十名母亲(37.0%)在婴儿被诊断之前没有被诊断出患有任何自身免疫性疾病。其中,三人随后被诊断为SLE,5人被诊断出患有干燥综合征,其中两人仍然没有任何自身免疫性疾病的诊断。发烧是NLE的常见症状;因此,当婴儿没有明确的发烧焦点时,NLE需要考虑,尤其是有皮疹的病例.
    Neonatal lupus erythematosus (NLE) is a rare disease caused by passively transmitted autoantibodies from the mother. NLE is a multi-organ system disease characterized by cutaneous, cardiac, hematological, hepatobiliary, and neurological manifestations. This study aimed to review the various symptoms and clinical manifestations in young infants with NLE and their mothers. We conducted a retrospective review of medical records of patients with NLE who were both examined and treated at Pusan National University Children\'s Hospital between January 2009 and December 2020 and their mothers. Twenty-seven patients with NLE comprising 13 male patients (48.1%) and 14 female patients (51.9%) were included. The most common symptom was rash (40.7%), followed by fever (25.9%), arrhythmia (14.8%), splenomegaly (11.1%), and intrauterine growth retardation (7.4%). Seven patients with fever had various organ system manifestations, including cutaneous (100%), hematological (71.4%), hepatobiliary (57.1%), and central nervous system (CNS; 28.6%) manifestations. Two of the febrile patients had aseptic meningitis. Cutaneous, cardiac, hematological, hepatobiliary, and CNS involvement were noted in 44.4%, 18.5%, 51.9%, 40.7%, and 22.2% of the patients, respectively. Systemic lupus erythematosus (SLE) was the most common maternal disease (14/27, 51.9%). Ten mothers (37.0%) had not been diagnosed with any autoimmune disease until their babies were diagnosed. Among them, three were subsequently diagnosed with SLE, five were diagnosed with the Sjögren\'s syndrome, and two of them still had no known diagnosis of any autoimmune disorder. Fever is a common symptom of NLE; thus, when there is no clear focus of fever in infants, NLE needs to be considered, especially in cases with skin rashes.
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  • 文章类型: Journal Article
    新生儿红斑狼疮(NLE)是一种自身免疫性疾病,以眶周红斑皮疹为特征。尽管已知炎症后色素沉着减退和毛细血管扩张是可能的后遗症,这些特征在肤色中可能特别明显。在这里,我们描述了两名皮肤有色的婴儿,其中眶周色素沉着和毛细血管扩张是诊断NLE的线索。
    Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by a periorbital erythematous rash. Although post-inflammatory hypopigmentation and telangiectasias are known possible sequelae, these features may be particularly noticeable in skin of color. Herein, we describe two infants with skin of color in whom periorbital hypopigmentation and telangiectasias were clues to the diagnosis of NLE.
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