CAJ | 학술논문

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。
목적：제고대신생인홍반랑창（neonatal lupus erythematosus，NLE）적인식。방법총결분석1990년1월지2013년6월북경협화의원19례 NLE 모영적림상표현、면역상관혈청、치료화수진정황，병복습상관문헌。결과19례 NLE 중태인2례，남영10례，녀영7례；출현피부손해자15례，혈액계통손해자9례，간공능이상자5례，심장수루자10례，중추신경계통수루자1례。환인균유항핵항체화항 SSA 항체화/혹항 SSB 항체양성。환인모친잉전진단위계통성홍반랑창자12례、간조종합정자2례。4례환인사용당피질격소치료，2례연합사용인면역구단백。수방6개월지17년，제2례인산외，소유환인균여정상인동무이。결론NLE 림상이일과성피진화선천성심장전도조체위돌출표현，역가견간장、혈액계통화중추신경계통수루，기증상수착자신항체소실가자유。환인모친이계통성홍반랑창다견，항 Ro/SSA 화（혹）항 La/SSB항체양성모친시발생 NLE 적위험인소。
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.