新生儿红斑狼疮12例临床特征和远期随访

目的探讨新生儿红斑狼疮（NLE）的临床特征及远期预后。方法总结分析1995年1月至2008年1月诊断为NLE患儿的临床表现、风湿免疫病相关血清学指标及随访情况。结果12例NLE患儿进入分析,男7例,女5例;起病年龄1～59 d。12例患儿均有皮肤狼疮损害,表现为不规则圆形或卵圆状红色或暗紫色皮疹,表面有鲜屑样变;血常规异常11例,表现为WBC、Hb或PLT降低;肝脏损害6例,表现为皮肤轻度黄染、肝脾肿大和ALT升高;心脏损害4例,表现为完全右束支传导阻滞、T波低平或左房轻度增大;ANA和抗SSA/Ro抗体阳性12例,抗SSB/La抗体阳性5例,抗ds-DNA抗体和抗U1-RNP抗体阳性各4例;ESR增快6例。12例患儿母亲ANA和抗SSA/Ro抗体均阳性,其中10例诊断为系统性红斑狼疮,2例诊断为干燥综合征。1例患儿于生后2周死亡,11例随访18个月至12年。随访至12月龄时,11例皮肤狼疮损害、肝脏损害和心脏损害均恢复正常,10例患儿风湿免疫病相关自身抗体转阴。1例3岁时诊断为幼年特发性关节炎少关节炎型,1例12岁时反复出现颜面红斑皮疹。结论NLE临床表现主要有皮肤狼疮损害、先天性心脏传导阻滞、血细胞减少、肝脾肿大和肝功能损害等,多于12个月内恢复。NLE患儿需长期随访,有发展为其他自身免疫性疾病的可能。

The clinical features and long time follow-up of neonatal lupus erythematosus in 12 children

Objective To investigate the the clinical features and long-term prognosis of neonatal lupus erythematosus （NLE） . Methods The clinical manifestations of patients and their mothers were summarized and analyzed. Their autoantibodies were detected, and long term follow-up was carried out. Results （1）12 NLE cases, 7 males and 5 females,all had lesions on skin. 11 cases had hematologic changes ,6 of them were with hepar impairments, and 4 cases had heart impairment. The antinuclear antibody and anti-SSA/Ro were positive in all cases. The anti-SSB/La was positive in 5 cases. The anti-dsDNA antibody was positive in 4 cases. Antibody against U1-RNP was positive in 4 cases, the level of ESR was higher in 6 cases. The alanine aminotransferase levels in 6 cases were higher than those in normal infants. （2） 11 mothers had no symptom before pregnancy, one had solar dermatitis. 7 mothers manifested solar dermatitis and light desquamation during the second trimester of pregnancy. 2 had fever, hair loss and cytopenia. 2 presented with rash and hair loss one month after delivery. The antinuelear antibody and anti-SSA/Ro antibody were positive in all mothers, the anti-SSB/La antibody was positive in 5 mothers, the anti-ds-DNA antibody was positive in 4 mothers, antibody against U1-RNP was positive in 4 mothers, the level of ESR was higher in 5 mothers. （3）Among the 12 cases, 11 cases were followed-up for 18 months to 12 years, one ease died from complete right bundle branch block. The hematologic changes and the hepatic impairment returned to normal, cutaneous lesions and autoantibodies disappeared about 6 to 12 months after birth in the 11 cases, at the time when maternal antibodies exhausted in the neonatal circulation. One case was diagnosed as JIA when he was 3 years old. Another case had recurrent rash when he was 12 years old, the ANA titer was 1：320 and the anti ds-DNA antibody was negative. Ten mothers were diagnosed as SLE and two mothers were Sjogren＇s syndrome. Conclusions The clinical manifestations of NLE include cardiac conduction disturbances, transient cutaneous lesions, cytopenia, hepatic impairment, etc. The long-term prognosis for children with NLE is still under investigation and some infants with NLE may progress to other autoimmune diseases later in the childhood.