19例新生儿红斑狼疮的心血管表现

doi:10.3969/j.issn.1000-8535.2022.06.010

摘要/Abstract

摘要： 目的总结新生儿红斑狼疮(NLE)的临床特征和预后,提高对NLE的认识。方法回顾分析2015年1月—2020年12月在广州市妇女儿童医疗中心住院诊治的19例NLE的临床资料,并进行文献复习。结果收集19例新生儿红斑狼疮,男10例,女9例。皮肤受累者19例;心血管系统受累者7例,其中心电生理异常7例:Ⅲ度房室传导阻滞(AVB)2例,交界性心律1例,Ⅱ度AVB 2例,完全性左束支传导阻滞(CLBBB)1例,T波低平1例;扩张型心肌病(DCM)1例;房间隔缺损1例。血液系统受累者3例;消化系统受累者7例。患儿抗SSA或抗SSB阳性18人,抗核抗体阳性18人,dsDNA阳性2人,抗RNP阳性3人。孕母产前诊断系统性红斑狼疮4例,干燥综合征8例,未分化结缔组织病1例,无任何临床症状6例(有无诊断疾病),其中10例予糖皮质激素治疗。随访时间5月~62月,随访18例。心血管系统受累者的预后差异大,2例Ⅲ度AVB予心脏起搏器治疗,1例交界性心律者建议安装起搏器。1例CLBBB并DCM者的内科治疗效果差。2例Ⅱ度AVB 者无明显临床症状。T波低平者复查为正常。随访中未发现患儿及其母亲有新发自身免疫性疾病。结论新生儿红斑狼疮临床表现以心血管系统受累为突出特征,其房室结外病变需引起关注。患儿和其母亲的长期随访非常重要。

关键词: 红斑狼疮, 新生儿, 皮疹, 心血管系统

Abstract: Objective To summarize the clinical features and prognosis characteristics of neonatal lupus erythematosus (NLE).Methods The clinical data of 19 cases of NLE who were hospitalized in Guangzhou Women and Children's Medical Center from January 2015 to December 2020 were retrospectively analyzed,and the literature was reviewed.Results A total of 19 cases of neonatal lupus erythematosus were enrolled,10 males and 9 females.There were 19 cases of cutaneous lesions; 7 cases of cardiac system involvement,including 7 cases of electrophysiological abnormalities: 2 cases of third-degree atrioventricular block (AVB),1 case of junctional heart rhythm,2 cases of second-degree AVB,1 case of complete left bundle branch block (CLBBB),1 case of low T-wave.Dilated cardiomyopathy(DCM)was found in 1 case,and atrial septal defect was found in 1 case.There were 3 cases of blood system abnormalities and 7 cases of digestive system problems.Eighteen children were positive for anti-SSA or anti-SSB test,18 were positive for ANA,2 were positive for dsDNA,and 3 were positive for anti-RNP.Four cases of mother was prenatal diagnosed with systemic lupus erythematosus,8 cases of Sjgren's syndrome,1 case of undifferentiated connective tissue disease,and 6 cases without any clinical symptoms,among them,10 cases were treated with glucocorticoids.The follow-up time varied from 5 months to 62 months,and 18 cases were followed up.The prognosis of patients with cardiac system involvement varied greatly.Two patients with third-degree AVB were treated with pacemaker,and 1 patient with junctional rhythm was recommended to install pacemaker.The patient with CLBBB and DCM had poor outcome with medical treatment.Two patients with second-degree AVB had no clinical symptoms,and the low T-wave one returned to normal.During the follow-up,no new autoimmune diseases were found in the children and their mothers.Conclusions The clinical manifestations of neonatal lupus erythematosus are prominently characterized by involvement of the cardiovascular system,and the extranodal lesion need to be paid attention to.Long-term follow-up of children and their mothers is critically important.

Key words: lupus erythematosus, newborn, rash, cardiac system

李珊, 李明, 张旭. 19例新生儿红斑狼疮的心血管表现[J]. 广州医药, 2022, 53(6): 48-51.

LI Shan, LI Ming, ZHANG Xu. Cardiovascular manifestations of 19 neonatal lupus erythematosus[J]. Guangzhou Medical Journal, 2022, 53(6): 48-51.

参考文献

[1] BUYON J P,CLANCY R M.Neonatal lupus syndromes[J].Curr Opin Rheumatol,2003,15(5):535-541.
[2] IZMIRLY P M,SAXENA A,KIM M Y,et al.Maternal and fetal factors associated with mortality and morbidity in a multi-racial/ethnic registry of anti-SSA/Ro-associated cardiac neonatal lupus[J].Circulation,2011,124(18):1927-1935.
[3] JAEGGI E T,HAMILTON R M,SILVERMAN E D,et al.Outcome of children with fetal,neonatal or childhood diagnosis of isolated congenital atrioventricular block[J].J Am Coll Cardiol,2002,39(1):130-137.
[4] BRITO-ZERÓN P,IZMIRLY P M,RAMOS-CASALS M,et al.The clinical spectrum of autoimmune congenital heart block[J].Nat Rev Rheumatol,2015,11(5):301-312.
[5] LEE L A,SOKOL R J,BUYON J P.Hepatobiliary disease in neonatal lupus: prevalence and clinical characteristics in cases enrolled in a national registry[J].Pediatrics,2002,109(1):E11.
[6] ACHERMAN R J,FRIEDMAN D M,BUYON J P,et al.Doppler fetal mechanical PR interval prolongation with positive maternal anti-RNP but negative SSA/Ro and SSB/La auto-antibodies[J].Prenat Diagn,2010,30(8):797-799.
[7] LIAO H,TANG C,QIAO L,et al.Prenatal management strategy for immune-associated congenital heart block in fetuses[J].Front Cardiovasc Med,2021(8):644122.
[8] BUYON J P,CLANCY R M,FRIEDMAN D M.Cardiac manifestations of neonatal lupus erythematosus: guidelines to management,integrating clues from the bench and bedside[J].Nat Clin Pract Rheumatol,2009,5(3):139-148.
[9] LI Y-Q,WANG Q,LUO Y,et al.Neonatal lupus erythematosus: a review of 123 cases in China[J].Int J Rheum Dis,2015,18(7):761-767.
[10] CAPONE C,BUYON J P,FRIEDMAN D M,et al.Cardiac manifestations of neonatal lupus: a review of autoantibody-associated congenital heart block and its impact in an adult population[J].Cardiol Rev,2012,20(2):72-76.
[11] AMBROSI A,SONESSON S-E,WAHREN-HERLENIUS M.Molecular mechanisms of congenital heart block[J].Exp Cell Res,2014,325(1):2-9.
[12] BOH E E.Neonatal lupus erythematosus[J].Clin Dermatol,2004,22(2):125-128.
[13] MIYAGAWA S.Neonatal lupus erythematosus: a review of the racial differences and similarities in clinical,serological and immunogenetic features of Japanese versus Caucasian patients[J].J Dermatol,2005,32(7):514-522.
[14] BUYON J P,CLANCY R M.From antibody insult to fibrosis in neonatal lupus - the heart of the matter[J].Arthritis Res Ther,2003,5(6):266-270.
[15] IZMIRLY P M,SAXENA A,SAHL S K,et al.Assessment of fluorinated steroids to avert progression and mortality in anti-SSA/Ro-associated cardiac injury limited to the fetal conduction system[J].Ann Rheum Dis,2016,75(6):1161-1165.
[16] MOAK J P,BARRON K S,HOUGEN T J,et al.Congenital heart block: development of late-onset cardiomyopathy,a previously underappreciated sequela[J].J Am Coll Cardiol,2001,37(1):238-242.
[17] RIVERA T L,IZMIRLY P M,BIRNBAUM B K,et al.Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus[J].Ann Rheum Dis,2009,68(6):828-835.
[18] MARTIN V,LEE L A,ASKANASE A D,et al.Long-term followup of children with neonatal lupus and their unaffected siblings[J].Arthritis Rheum,2002,46(9):2377-2383.