Cimaz Rolando, Spence Dawn L, Hornberger Lisa, Silverman Earl D
Pediatric Department, ICP, Milano, Italy.
J Pediatr. 2003 Jun;142(6):678-83. doi: 10.1067/mpd.2003.233.
Neonatal lupus erythematosus (NLE) is characterized by complete congenital heart block (CCHB), cutaneous rash, and laboratory abnormalities in infants born to mothers with autoantibodies directed against SSA/Ro, SSB/La, or both. We carried out a prospective study to determine the incidence of individual NLE features.
The study was performed in two centers: Toronto, Canada, and Milano, Italy. Mothers had been referred for the presence of anti-SSA/Ro autoantibodies, regardless of their diagnosis. All the children were seen at least once within the first 6 months of life for clinical evaluation and laboratory testing. The study group consisted of 128 infants born from 124 pregnancies in 112 women with anti-Ro antibodies with or without anti-La antibodies.
There were two cases of CCHB for an overall percentage of 1.6%. Twenty-one children (16%) developed cutaneous NLE. Laboratory testing showed hematologic abnormalities in 27% of the babies and elevation of liver enzymes in 26%.
Mothers with autoimmune diseases and anti-Ro antibodies are at risk of delivering a child with NLE but at a low risk of delivering a child with CCHB. Infants born to mothers with anti-Ro or anti-La antibodies should be monitored for other features of NLE in addition to CCHB.
新生儿红斑狼疮(NLE)的特征为完全性先天性心脏传导阻滞(CCHB)、皮疹以及实验室检查异常,见于母亲携带抗SSA/Ro、抗SSB/La自身抗体或两者皆有的婴儿。我们开展了一项前瞻性研究以确定NLE各特征的发生率。
该研究在两个中心进行:加拿大的多伦多和意大利的米兰。母亲因存在抗SSA/Ro自身抗体而被转诊,无论其诊断情况如何。所有儿童在出生后的前6个月内至少接受过一次临床评估和实验室检查。研究组由112名携带抗Ro抗体(有或无抗La抗体)的女性所怀124次妊娠分娩的128名婴儿组成。
有2例CCHB,总体发生率为1.6%。21名儿童(16%)出现皮肤型NLE。实验室检查显示,27%的婴儿有血液学异常,26%的婴儿肝酶升高。
患有自身免疫性疾病且携带抗Ro抗体的母亲有分娩患NLE儿童的风险,但分娩患CCHB儿童的风险较低。除CCHB外,对携带抗Ro或抗La抗体母亲所生婴儿还应监测NLE的其他特征。