Li Xiaoxia, Huang Xianmei, Lu Hui
Department of Neonatal Intensive Care Unit.
Department of Pediatrics, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Medicine (Baltimore). 2018 Nov;97(45):e13185. doi: 10.1097/MD.0000000000013185.
Neonatal lupus erythematosus (NLE) is an infrequent disease caused by transplacental maternal autoantibodies. The most common effects of NLE include cutaneous involvement and congenital heart block (CHB), although it might involve multiple organs, such as the liver, lungs, blood, and nervous or digestive systems. Izmirly PM1 and Tonello et al recently reported cutaneous manifestations of neonatal lupus and risk of subsequent CHB. The most serious complication of NLE is complete atrioventricular (AV) block.
We experienced 2 cases of NLE that were diagnosed in the past year in our Neonatal Intensive Care Unit. These cases showed 2 different clinical spectrums (CHB, multisystemic effects). One case was a 32-week pregnant woman with combined liver damage and fever, and her fetus was premature due to bradycardia and pericardial effusion. The second case was a young pregnant woman who had systemic lupus erythematosus for 2 years and had been taking methylprednisolone and hydroxychloroquine for a long time since her illness. When prenatal testing at 28 weeks of pregnancy showed that the fetus had CHB, the mother began taking dexamethasone.
The first case was diagnosed as NLE with CHB after birth, while the second was diagnosed as NLE with CHB, ductus arteriosus, and atrial septal defect when she was born at 34 weeks.
Both of 2 cases were treated with steroids, intravenous immunoglobulin, and a diuretic. But the second case was treated with isoprenaline in addition to the above.
Both of the infants was followed up and found to be clinically normal. During the clinic follow-up of the first case, the 8-month-old infant was still asymptomatic with normal growth and development. Her heart rate fluctuated from 40 to 90 beats/minute.
Autoimmune CHB is a severe, potentially life-threatening disorder associated with passive transfer of maternal anti-Sjogren's syndrome A/Ro and anti-Sjogren's syndrome B/La autoantibodies. Mothers who are positive for these autoantibodies are recommended to have serial echocardiography and obstetric ultrasonography from the early second trimester. Newborns should be delivered at an early stage of gestation if there is evidence of pericardial effusion, ascites, increasing ventricular ectopy, reduced ventricular shortening fraction, or AV valve regurgitation. Aggressive medical management after birth should be coupled with pacemaker implantation in infants who do not respond to medical therapies alone.
新生儿红斑狼疮（NLE）是一种由经胎盘传递的母体自身抗体引起的罕见疾病。NLE最常见的影响包括皮肤受累和先天性心脏传导阻滞（CHB），尽管它可能累及多个器官，如肝脏、肺、血液以及神经或消化系统。Izmirly PM1和Tonello等人最近报道了新生儿狼疮的皮肤表现及随后发生CHB的风险。NLE最严重的并发症是完全性房室传导阻滞。
我们在新生儿重症监护病房去年诊治了2例NLE病例。这些病例呈现出两种不同的临床谱（CHB、多系统受累）。一例是一名32周妊娠的孕妇，合并肝损伤和发热，其胎儿因心动过缓和心包积液而早产。另一例是一名年轻孕妇，患有系统性红斑狼疮2年，自患病以来长期服用甲泼尼龙和羟氯喹。妊娠28周进行产前检查时发现胎儿患有CHB，母亲开始服用地塞米松。
第一例出生后被诊断为患有CHB的NLE，而第二例在34周出生时被诊断为患有CHB、动脉导管未闭和房间隔缺损的NLE。
两例均接受了类固醇、静脉注射免疫球蛋白和利尿剂治疗。但第二例除上述治疗外还接受了异丙肾上腺素治疗。
两名婴儿均接受随访，临床检查均正常。在对第一例进行门诊随访期间，该8个月大的婴儿仍无症状，生长发育正常。其心率在40至90次/分钟之间波动。
自身免疫性CHB是一种严重的、可能危及生命的疾病，与母体抗干燥综合征A/Ro和抗干燥综合征B/La自身抗体的被动传递有关。建议这些自身抗体呈阳性的母亲从孕中期早期开始进行系列超声心动图和产科超声检查。如果有证据表明存在心包积液、腹水、室性早搏增加、心室缩短分数降低或房室瓣反流，新生儿应在妊娠早期分娩。出生后积极的药物治疗应与对单独药物治疗无反应的婴儿进行起搏器植入相结合。
