摘要
目的总结新生儿红斑狼疮(neonatal lupus erythematosus, NLE)的临床特点,以提高临床对其的认识。 方法回顾性纳入福建省妇幼保健院新生儿科2010年9月至2017年9月收治的13例NLE患儿,总结其临床表现、实验室检查、治疗和随访情况,并复习相关文献。采用描述性统计分析。 结果13例NLE患儿中,男8例,女5例;皮肤损害8例,心脏损害7例(6例表现为房室传导阻滞),血液系统损害7例,肝胆系统损害3例,中枢神经系统和肾功能损害各1例。13例患儿的抗核抗体和抗干燥综合征A抗原抗体均为阳性,抗干燥综合征B抗原抗体阳性11例,抗双链DNA抗体阳性2例。13例患儿母亲中3例孕前诊断干燥综合征,2例孕前诊断系统性红斑狼疮,2例孕期诊断干燥综合征,1例孕期诊断系统性红斑狼疮。8例皮肤损害患儿采取避光治疗,生后1~6个月皮疹陆续消退;5例血小板减少患儿给予静脉注射人免疫球蛋白治疗,1例贫血患儿给予输注悬浮红细胞治疗,血液系统损害于生后2~3个月内逐步恢复正常;3例肝功能异常患儿给予保肝、退黄等对症治疗,生后2~6个月内恢复正常。13例患儿随访5个月至7年,其中7例病情好转,生长发育正常,5例仍为Ⅲ度房室传导阻滞,1例患儿在11月龄时安装心脏起搏器。3例孕期无症状母亲在患儿被诊断为NLE后随访发现有自身免疫性疾病,1例为干燥综合征,2例为系统性红斑狼疮。 结论NLE以皮肤损害和先天性心脏传导阻滞为主要表现,肝脏、血液和中枢神经系统等亦可受累。对具有高危因素的母子,要及时完善自身免疫性抗体等检查,以便于早期诊断及干预,并需要长期随访。
ObjectiveTo summarize the clinical features of neonatal lupus erythematosus (NLE) and to have a better clinical understanding. MethodsWe retrospectively analyzed the clinical data of 13 patients with NLE who were hospitalized in Fujian Provincial Maternity and Children's Hospital from September 2010 to September 2017. The pathogenesis, clinical manifestations, laboratory examinition, management and long term outcomes of these babies were summarized, and the relevant literatures were also reviewed. Descriptive statistical analysis was performed. ResultsThe 13 NLE cases included eight boys and five girls. Among them, skin lesions, cardiac impairment, hematological problems, hepatobiliary system damage, central nervous system involvement and renal function damage occurred in eight, seven (six cases were atrioventricular block), seven, three, one and one case, respectively. Antinuclear antibodies and anti-Sjogren's syndrome antigen A antibodies were positive in all neonates, anti-Sjogren's syndrome antigen B antibodies were positive in 11, and anti-double-stranded DNA antibodies were positive in two cases. Among the 13 mothers, three were diagnosed with Sjogren's syndrome and two had systemic lupus erythematosus(SLE) before pregnancy, two were diagnosed with Sjogren's syndrome and one developed SLE during pregnancy. Eight babies with skin lesions were asked to avoid light and the skin rash all gradually receded within 1-6 months after birth. Five cases with thrombocytopenia were treated with intravenous immunoglobulin and one anemic baby received erythrocyte transfusion. Within 2 to 3 months, the impaired blood system in these babies were back to normal. For the three babies with abnormal liver functions, hepatic protectants and jaundice relieving agents were given, and 2 to 6 months later they recovered. The 13 patients were followed up for five months to seven years, among which, seven improved with normal growth and development;five still had grade Ⅲ atrioventricular block;one installed an atrial pacemaker at 11 months. Three mothers who were asymptomatic during pregnancy were found to have autoimmune diseases after their babies were diagnosed with NLE, including one case of Sjogren's syndrome and two of SLE. ConclusionsNLE is mainly characterized by skin lesions and congenital heart block, while liver, blood system, central nervous system and other organs may also be involved. For high-risk mothers and babies, timely autoantibody screening and relevant examinations are suggested for early diagnosis and interventions. In addition, long term follow-up is required for affected cases.
作者
郑义熔
杨长仪
修文龙
林佳佳
Zheng Yirong;Yang Changyi;Xiu Wenlong;Lin Jiajia(Department of Neonatology,Fujian Provincial Maternity and Children's Hospital,Affiliated Hospital of Fujian Medical University,Fuzhou 350001,China)
出处
《中华围产医学杂志》
CAS
CSCD
北大核心
2019年第4期278-283,共6页
Chinese Journal of Perinatal Medicine
关键词
红斑狼疮
系统性
婴儿
新生
Lupus erythematosus, systemic
Infant, newborn
