CAJ | 학술논문

目的：探讨新生儿红斑狼疮（NLE）的临床特点及远期预后，提高对 NLE 的认识。方法总结分析2010年6月至2014年1月诊断为 NLE 患儿的临床表现、相关血清学指标等辅助检查。定期随访患儿一般情况、皮疹、血常规、尿常规、肝肾功能、补体、红细胞沉降率（ESR）、自身抗体、心电图、超声心动图。结果共11例患儿诊断为 NLE，男6例，女5例。临床表现：11例均有皮肤狼疮损害，血小板减少3例，肝损害7例，心脏损害4例。抗干燥综合征 A 抗原（SSA/ Ro）抗体和抗核抗体（ANA）均阳性11例，抗干燥综合征 B 抗原抗体阳性5例，抗双链-DNA 抗体阳性4例，抗 U1核糖核蛋白抗体阳性3例，ESR 增快5例。11例患儿母亲 ANA 和抗SSA/ Ro 抗体均阳性，其中7例诊断为系统性红斑狼疮，3例诊断为干燥综合征，1例孕前无症状。7例患儿予保肝降酶治疗，其中3例接受糖皮质激素治疗，1例联合静脉用丙种球蛋白治疗。10例患儿随访10个月~4年，1例完全性房室传导阻滞患儿于出生5周死亡。随访至1岁时，10例皮肤狼疮损害、肝损害均恢复正常，9例患儿风湿免疫相关自身抗体转阴，1例1岁时诊断川崎病。结论 NLE 临床表现皮肤损害最常见，血液系统和肝脏异常常见，但一般不严重，心脏病变尤其是完全性房室传导阻滞少见。NLE 患儿需长期随访，多数预后良好，少数有发展为其他自身免疫性疾病的可能。
목적：탐토신생인홍반랑창（NLE）적림상특점급원기예후，제고대 NLE 적인식。방법총결분석2010년6월지2014년1월진단위 NLE 환인적림상표현、상관혈청학지표등보조검사。정기수방환인일반정황、피진、혈상규、뇨상규、간신공능、보체、홍세포침강솔（ESR）、자신항체、심전도、초성심동도。결과공11례환인진단위 NLE，남6례，녀5례。림상표현：11례균유피부랑창손해，혈소판감소3례，간손해7례，심장손해4례。항간조종합정 A 항원（SSA/ Ro）항체화항핵항체（ANA）균양성11례，항간조종합정 B 항원항체양성5례，항쌍련-DNA 항체양성4례，항 U1핵당핵단백항체양성3례，ESR 증쾌5례。11례환인모친 ANA 화항SSA/ Ro 항체균양성，기중7례진단위계통성홍반랑창，3례진단위간조종합정，1례잉전무증상。7례환인여보간강매치료，기중3례접수당피질격소치료，1례연합정맥용병충구단백치료。10례환인수방10개월~4년，1례완전성방실전도조체환인우출생5주사망。수방지1세시，10례피부랑창손해、간손해균회복정상，9례환인풍습면역상관자신항체전음，1례1세시진단천기병。결론 NLE 림상표현피부손해최상견，혈액계통화간장이상상견，단일반불엄중，심장병변우기시완전성방실전도조체소견。NLE 환인수장기수방，다수예후량호，소수유발전위기타자신면역성질병적가능。
Objective To investigate the clinical features and long - term prognosis of neonatal lupus erythe-matosus(NLE)and to improve the understanding of NLE. Methods The clinical manifestations and related serologi-cal tests of NLE children diagnosed from June 2010 to January 2014 were analyzed. Regular follow - up was carried out to detect the general condition,rash,blood routine,urine routine,liver and kidney function,complement,red blood cell sedimentation rate(ESR),auto antibodies,electrocardiogram,and ultrasound cardiogram. Results Among the 11 NLE cases,there were 6 male and 5 female patients. All had lesions on skin,3 cases had hematologic changes,7 cases were had liver damage,and 4 cases had heart impairment. The antinuclear antibody and anti - sjogren sydrome A/ Ro antigen (SSA/ Ro)were positive in all the patients. The anti - sjogren sydrome B antigen was positive in 5 patients. The anti -double stands deoxyribonucleic acid antibody was positive in 4 patients. Antibody against U1 - ribonudeoprotein was positive in 3 patients,and the level of ESR was higher in 5 patients. The antinuclear antibody and anti - SSA/ Ro anti-body were positive in all mothers. Only 1 mother had no symptom before pregnancy,7 patients had SLE,3 patients had sjogren syndrome. Seven patients received protect liver enzyme treatment,3 cases of glucocorticoid therapy,and 1 case had combined intravenous treatment with gamma globulin. Among the 11 cases,10 cases were followed up for 10 months to 4 years,while 1 case died from complete bundle branch block after 5 weeks of birth. At 1 year old,10 cases of cuta-neous lupus damage had liver damage were resorted to normal,and the rheumatic autoimmune related autoantibodies of 9 cases turned to be negative,but 1 case was diagnosed as Kawasaki disease when she was 1 year old. Conclusions One of the most common clinical manifestations of NLE was damage of skin,had the liver and blood system abnormity were common but usually not serious. Heart disease especially complete atrioventricular block was less. The long - term follow - up for children with NLE is necessary,and the majority of the prognosis is good,as only a few have the possibi-lity of developing other autoimmune diseases.