CAJ | 학술논문

目的提高对新生儿红斑狼疮(NLE)的认识.方法报道1990年至今广东省人民医院7例患儿和母亲,并复习1980年以来国内文献报道的87例,总结其发病机制、临床表现、自身抗体、治疗及随访情况.结果共94例NLE患儿.男∶女=48∶46.皮疹73例.心脏损害23例,其中心电图异常20例,Ⅲ度房室传导阻滞(AVB)仅2例,结构异常9例.血液系统损害44例,肝脏损害30例,肾脏病变3例,肌酶升高3例,神经系统异常2例.患儿中86例抗SSA阳性(91.5%),51例抗SSB阳性(54.3%),16例抗ds-DNA阳性,14例抗U1-RNP抗体阳性,13例抗Sm阳性,6例抗RNP阳性,4例抗rRNP阳性.患儿母亲孕前无症状39例(占44.8%),孕前和孕期新发红斑狼疮共48例(51.1%),干燥综合征(SS)3例,光过敏、关节痛及冻疮各2例,类风湿关节炎(RA)1例,牛皮癣1例.20例患儿接受了糖皮质激素治疗,其中4例联合静脉用丙种球蛋白治疗.随访68例,最长者达12年,其中健康58例,病情好转5例,3例死亡,2例仍为Ⅲ度AVB,但未安装起搏器.结论 NLE是一种少见的获得性自身免疫性疾病,其母亲以患红斑狼疮者最多,孕前无症状者占44.8%.我国NLE以一过性的皮肤损害最常见,心脏病变尤其是Ⅲ度AVB少见,肝脏和血液系统异常常见,但一般不严重.抗SSA和(或)抗SSB抗体是患儿及母亲最常见的自身抗体.多数患儿预后相对良好.
목적 제고대신생인홍반랑창(NLE)적인식.방법 보도1990년지금광동성인민의원7례환인화모친,병복습1980년이래국내문헌보도적87례,총결기발병궤제、림상표현、자신항체、치료급수방정황.결과 공94례NLE환인.남∶녀=48∶46.피진73례.심장손해23례,기중심전도이상20례,Ⅲ도방실전도조체(AVB)부2례,결구이상9례.혈액계통손해44례,간장손해30례,신장병변3례,기매승고3례,신경계통이상2례.환인중86례항SSA양성(91.5%),51례항SSB양성(54.3%),16례항ds-DNA양성,14례항U1-RNP항체양성,13례항Sm양성,6례항RNP양성,4례항rRNP양성.환인모친잉전무증상39례(점44.8%),잉전화잉기신발홍반랑창공48례(51.1%),간조종합정(SS)3례,광과민、관절통급동창각2례,류풍습관절염(RA)1례,우피선1례.20례환인접수료당피질격소치료,기중4례연합정맥용병충구단백치료.수방68례,최장자체12년,기중건강58례,병정호전5례,3례사망,2례잉위Ⅲ도AVB,단미안장기박기.결론 NLE시일충소견적획득성자신면역성질병,기모친이환홍반랑창자최다,잉전무증상자점44.8%.아국NLE이일과성적피부손해최상견,심장병변우기시Ⅲ도AVB소견,간장화혈액계통이상상견,단일반불엄중.항SSA화(혹)항SSB항체시환인급모친최상견적자신항체.다수환인예후상대량호.
Objective To acquire more knowledge about neonatal lupus erythematosus (NLE).Method Seven cases with neonatal lupus erythematosus who were seen in this hospital from 1990 to 2009 are reported in this paper and 87 cases reported previously from 1980 to now in China were reviewed. The clinical manifestations, serum autoantibodies, treatment and results of long-term follow-up are analyzed and summarized. Result Totally 94 cases were summarized. Male/female ratio was 48/46; 73 cases had skin rash; 23 had heart abnormality, among whom 13 had cardiac conductive problems including 8 cases of atrioventricular blockage (AVB) (3 degree Ⅰ , 3 degree Ⅱ and 2 degree Ⅲ ) and 5 cases of right bundle branch block cases (RBBB). Nine cases had anatomical abnormality including 5 cases of atrial septal defect (ASD), 2 cases of ventricular septal defect (VSD) and 2 cases of atrial enlargements. Forty-four cases had hematological problems including 28 with thrombocytopenia, 11 with leukocytopenia and 34 with anemia.Thirty cases had hepatic abnormality, including 24 liver dysfunction, 22 hepatomegaly, 6 splenomegaly and 3 cholestasis. Three cases had nephropathy; 3 had elevated creatine kinase; 2 had nervous disorder. Among the 94 cases, 86 (91.5%) were positive for anti-SSA, 51 (54. 3% ) anti-SSB, 16 anti-ds-DNA, 14 antiU1 -RNP, 13 anti-Sm ( + ), 6 anti-RNP and 4 anti-rRNP( + ). Among the corresponding mothers, 39 cases (44. 8% ) were asymptomatic before pregnancy, 35 had SLE, 5 had SCLE, 3 had Sjogren syndrome, 2 had chilblain, photosensitivity and arthralgia, respectively, 1 had rheumatoid arthritis and 1 had psoriasis.During pregnancy, 8 mothers developed SLE. Totally 48 mothers ( 51.1% ) suffered from LE. Together with 15 mothers who had transient skin rash during the pregnancy, there were 23 mothers (59%) who had new clinical manifestation among the 39 asymptomatic mothers. Twenty NLE cases accepted glucocorticoid treatment, 4 of them were treated with intravenous immunoglobulin. Sixty-eight cases were followed up for upto 12 years, 58 cases were healthy, 5 cases improved and 3 died. Two cases still had grade Ⅲ AVB without pacemaker. Conclusion NLE is a rare acquired autoimmune disease. Although nearly half of the mothers were asymptomatic before pregnancy, more than half of them developed LE or other symptoms. The clinical presentations in Chinese cases include a transient rash, cardiac lesion while grade Ⅲ AVB was rare,hematological changes and liver impairments which were common but not serious. Anti-SSA and/or anti-SSB were the most related autoantibody. Most patients with NLE have relatively good prognosis.