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目的探讨新生儿红斑狼疮(neonatal lupus erythematosus,NLE)临床特点、治疗及预后。方法回顾性分析本科诊治的4例新生儿红斑狼疮临床资料,并复习相关文献。结果男女各2例,皮损均主要位于头面及躯干,无心脏传导阻滞,仅1例心脏B超示:先天性心脏病,房间隔中心型缺损(房水平左向右分流);2例有轻度至中度贫血;1例出生后10天时出现转氨酶升高及高胆红素血症。患儿和其母亲抗核抗体(antinuclear antibody,ANA)及抗干燥综合征抗原A(SSA)抗体均阳性,3例抗干燥综合征抗原B(SSB)抗体阳性;1例患儿母亲产前诊断为干燥综合征(SS),1例母亲产后1月出现亚急性皮肤型红斑狼疮(subacute cutaneous lupus erythematosus,SCLE)皮损,另1例母亲产后2年诊断系统性红斑狼疮(systemic lupus erythematosus,SLE)。结论国内NLE患儿以一过性皮肤损害为主要临床表现,可伴有轻度肝脏和血液系统损害,而先天性心脏传导阻滞(congenital heart block,CHB)较为少见。抗Ro/SSA、抗La/SSB抗体是NLE患儿及母亲最常见的抗体。本病预后较好,但需长期随访。
Objective To investigate the clinical features, treatment and prognosis of neonatal lupus erythematosus (NLE). Methods Retrospective analysis of 4 cases of neonatal lupus clinical data, and review the relevant literature. Results There were 2 males and 2 females. The lesions were mainly located on the cephalic face and trunk with no heart block. Only 1 patient had congenital heart disease with congenital heart disease and atrial septal defect (left to right shunt), 2 patients Mild to moderate anemia; 1 case of elevated transaminase and hyperbilirubinemia occurred 10 days after birth. The antinuclear antibody (ANA) and anti-Sjogren’s syndrome A (SSA) antibodies were positive in both children and their mothers, and 3 were positive for anti-Sjogren syndrome B (SSB) antibody. One patient had a prenatal diagnosis of mother In the case of Sjogren’s syndrome (SS), 1 patient had subcutaneous lesions of subacute cutaneous lupus erythematosus (SCLE) in January and another 1 patient diagnosed systemic lupus erythematosus (SLE) 2 years after delivery. ). Conclusion The main clinical manifestations of transient skin lesions in children with NLE may be mild liver and blood system damage, while congenital heart block (CHB) is rare. Anti-Ro / SSA, anti-La / SSB antibodies are the most common antibodies in children and mothers with NLE. The prognosis of the disease is better, but long-term follow-up.