新生儿红斑狼疮临床特点及远期预后

Clinical features and long-term prognosis of neonatal lupus erythematosus

目的探讨新生儿红斑狼疮(NLE)的临床特点及远期预后。方法选择2011年4月至2021年12月中国人民解放军总医院第七医学中心新生儿重症监护室收治的病例资料完整的9例NLE患儿(患儿1~9)为研究对象。采用回顾性分析方法,对其病史,入院时实验室、心电图、心脏彩色多普勒超声检查结果等进行分析,并通过电话及门诊随访患儿出院后情况。本研究遵循的程序符合中国人民解放军总医院伦理委员会规定,并通过该委员会审批(伦理批号:2022-192),患儿监护人均签署临床研究知情同意书。结果①患儿1~9中,男性患儿为7例,女性为2例;出生胎龄为30~40+2周,足月儿为4例,起病年龄为生后1 h至17 d;首发症状为全身淤斑、皮疹及血小板计数(PLT)降低。②心电图检测结果示,1例(患儿2)为Ⅲ度房室传导阻滞(AVB)并交界性逸搏心律。2例(患儿3、9)心电轴右偏,其中患儿9合并右心室高电压。③心脏彩色多普勒超声检测结果显示,卵圆孔未闭(PFO)为8例(患儿1、3、4、5~9),动脉导管未闭为1例(患儿9),1例室间隔和左心室壁运动不协调,左心室射血分数为50%。④患儿1~9抗干燥综合征抗原A(SSA/Ro)抗体均呈阳性,抗干燥综合征抗原B(SSB/La)抗体呈阳性为3例(患儿1、3、5)。⑤对PLT降低的5例NLE新生儿(患儿1、4、5~7)采取静脉输注丙种球蛋白[1.0 g/kg×(1~2)次/d]治疗后,PLT水平均恢复正常。其中,1例(患儿2),同时采取放置心脏起搏器治疗,1例(患儿4)同时被诊断为湿疹+血小板减少伴免疫缺陷综合征(WAS)。⑥患儿1~9中,末次随访年龄最大为10岁(患儿6),最小为11个月龄(患儿4)。对患儿1~9均于生后6个月时查体的结果提示运动、智力、体力发育均正常。结论NLE新生儿多以皮疹、淤斑为首发症状,易导致心脏传导系统及血液系统损伤,并且同时合并自身抗SSA/Ro抗体和(或)抗SSB/La抗体、抗SSA/Ro-52抗体呈阳性。除心脏损害外,NLE新生儿临床表现多为暂时性,不需免疫抑制剂治疗的,该类新生儿整体预后良好。

Objective To study clinical characteristics and long-term prognosis of neonatal lupus erythematosus(NLE).Methods Nine children with NLE who were admitted to the Neonatal Intensive Care Unit of the Seventh Medical Center of the Chinese PLA General Hospital from April 2011 to December 2021 were selected into this study.The medical history,laboratory examination results,electrocardiogram and color Doppler echocardiography of these 9 NLE children were analyzed retrospectively,and the children were followed up by telephone and outpatient service.The procedure followed in this study conforms to the regulations of the Ethics Committee of Chinese PLA General Hospital(Approval No.2022-192),and informed consents were obtained from the guardians of children.Results①Among 9 children with NLE,7 were boy,with a gestational age ranging from 30 to 40+2 weeks.There were 4 full-term infants,and the age of onset ranged from 1 hour to 17 days.The initial symptoms included ecchymosis,rash,and decreased platelet count(PLT).②Electrocardiogram results showed that one child had third-degree atrioventricular block(AVB)with junctional escape rhythm.Two children had rightward deviation of the electrocardiographic axis,and one of them had high voltage in the right ventricle.③Color Doppler echocardiography showed that 8 cases had patent foramen ovale(PFO),1 case had patent ductus arteriosus(PDA),1 case had uncoordinated movement of the ventricular septum and left ventricular wall,and the left ventricular ejection fraction was 50%.④All 9 children tested positive for anti-Sjögren′s syndrome A(SSA/Ro),and 3 of them tested positive for anti-Sjögren′s syndrome B(SSB/La)antibody.⑤For the 5 children with decreased PLT,intravenous infusion of gamma globulin[1.0 g/kg×(1-2)times/d]was administered,and the PLT levels returned to normal.Among them,one child had a cardiac pacemaker placed,one was diagnosed with Wiskott-Aldrich syndrome(WAS)with eczema and thrombocytopenia,and the remaining children were normal.⑥Among the 9 children,the maximum follow-up age was 10 years old,and the minimum was 11 months.All 9 children were re-examined at 6 months after birth,and the results suggested that their motor,intellectual,and physical development were normal.Conclusions The first symptom of NLE is rash and ecchymosis,which is easy to lead to damage of cardiac conduction system and blood system.Positive autoantibodies against SSA/Ro and/or SSB/La and SSA/Ro-52 antibody are common.Except for heart damage,the clinical manifestations of NLE are temporary and do not require immunosuppressive treatment.The overall recovery is good,but long-term follow-up is still needed.