【摘要】 目的提高对新生儿红斑狼疮(neonatal lupus erythematosus,NLE)的认识。方法总结分析1990年1月至2013年6月北京协和医院19例NLE母婴的临床表现、免疫相关血清、治疗和随诊情况,并复习相关文献。结果 19例NLE中胎儿2例,男婴10例,女婴7例;出现皮肤损害者15例,血液系统损害者9例,肝功能异常者5例,心脏受累者10例,中枢神经系统受累者1例。患儿均有抗核抗体和抗SSA抗体和或抗SSB抗体阳性。患儿母亲孕前诊断为系统性红斑狼疮者12例、干燥综合征者2例。4例患儿使用糖皮质激素治疗,2例联合使用人免疫球蛋白。随访6个月至17年,除2例引产外,所有患儿均与正常儿童无异。结论 NLE临床以一过性皮疹和先天性心脏传导阻滞为突出表现,亦可见肝脏、血液系统和中枢神经系统受累,其症状随着自身抗体消失可自愈。患儿母亲以系统性红斑狼疮多见,抗RoSSA和(或)抗LaSSB抗体阳性母亲是发生NLE的危险因素。更多还原

【Abstract】 Objective The main purpose of this study was to improve the understanding of the pathogenesis and clinical manifestations of neonatal lupus( NL). Methods All the NL patients,who were hospitalized in the Peking Union Medical College Hospital from Jan 1990 to June 2013,were included in this study. Their clinical manifestations, serum auto-antibodies, treatment and results of long-term follow-up were analyzed. Moreover,the related literature was reviewed. Results A total of 19 cases of NL were included( 2 were fetus,10 were male newborn and 7 were female newborn). Among them,15 cases had skin rash,9 cases had hematological problems,5 cases had liver dysfunction,8 cases had heart abnormality,1 case had nervous disorder. All of them were positive for ANA and anti-SSA or anti-SSB. The mothers of 12 cases were diagnosed as systemic lupus erythematosus and 1 mother was diagnosed as Sjogren’s syndrome before pregnancy. Four cases accepted glucocorticoid treatment and 2 cases were treated with intravenous immunoglobulin. The follow-up time ranged from 6 months to 17 years. All children were normal.Conclusion The typical presentations of NL include transient skin rash and congenital heart block.Hematological changes, liver impairments and nervous disorder can also occur. All the symptoms can recover spontaneously along with the disappearance of autoantibodies.更多还原