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(Neonatal lupus erythematosus NLE)的发病机制、血清学改变、临床特征及远期预后。方法 确定NLE的条件：① 新生儿先天性心脏传导阻滞和新生儿或母亲的抗SSA/Ro、抗SSB/La抗体阳性；② 确定的和NLE相关的皮肤损害和新生儿或母亲的抗-Ro/SSA, 抗-La/SSB,或 抗-U1RNP抗体阳性。总结分析12例NLE患儿和母亲的临床表现，检测母亲和患儿的自身抗体，进行长期随访。并进行文献复习。结果 12例NLE , 男7例, 女5例, 就诊年龄1个月～5个月，起病年龄生后1d ～2个月，皮肤损害12例，血液学改变11例，肝脏受损6例，心脏受损4例，抗核抗体阳性12例，抗SSA/Ro抗 体阳性12例，抗SSB/La抗体阳性5例，抗ds-DNA抗体阳性4例，抗ENA抗体阳性4例，血沉增快6 例，血清丙氨酸转氨酶增高6例。12例母亲中孕前11例无症状，1例日光性皮炎；孕3～5个月时7例母亲出现日光性皮炎；2例发热、脱发、全血细胞减少；产后1个月 2例母亲出现皮疹、脱发；抗核抗体阳性12例，抗SSA/Ro抗体阳性12例，抗 SSB/La抗体阳性 5例，抗ds-DNA抗体阳性4例，抗ENA抗体阳性4例；血沉增快5例。12例NLE中11例随访18个月～12年，心电图完全右束支传导阻滞1例死亡。11例皮疹于3~11个月龄消退，自身抗体于6~12个月龄转阴，血液学改变、肝脏损害恢复正常，智力发育同正常同龄儿。1例3岁确诊为幼年特发性关节炎少关节炎型，1例12岁时出现反复颜面红斑皮疹，ANA阳性1：320，抗ds-DNA抗体阴性。12 例母亲中10例确诊为SLE，2例确诊为SS。结论 NLE是获得性的自身免疫疾病，临床表现主要有短暂性皮肤狼疮损害、先天性心脏传导阻滞、血细胞减少、黄疸、肝功能损伤等，肾脏受损少见。抗Ro (特别是抗-52KD SSA/Ro) 或抗-La抗体阳性的母亲是NLE的危险因素。NLE患儿需要长期随访，这些患儿存在到儿童期发展为其他自身免疫病的危险。

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

ObjectiveTo investigate the the clinical features and long-term prognosis of neonatal lupus erythematosus (NLE) .MethodsThe clinical manifestations of patients and their mothers were summarized and analyzed.Their autoantibodies were detected, and long term follow-up was carried out.Results①12 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.②11 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 antinuclear 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. ③Among the 12 cases, 11 cases were followed-up for 18 months to 12 years,one case 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.ConclusionsThe 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.