Abstract:
Neonatal lupus erythematosus (NLE) is a congenital autoimmune condition in which the transplacental passage of immunoglobulin G (IgG) directed against auto-antigens causes clinical symptoms in the foetus or neonate. Anti-Ro/SS-A, anti-La/SS-B, and to a lesser extent, anti-U1RNP autoantibodies (aAbs) have the strongest association with NLE. However, ~ 50% of affected mothers are asymptomatic despite carrying those aAbs. The clinical picture of the disease is very diverse. Cardiac manifestations are the most severe, including congenital heart block (CHB), with a mortality rate of ~18%. Preventative therapy with hydroxychloroquine (HCQ) reduces the recurrence rate of CHB in subsequent pregnancies by ~50%.
摘要:
新生儿红斑狼疮(NLE)是一种先天性自身免疫性疾病,其中针对自身抗原的免疫球蛋白G(IgG)经胎盘通过会引起胎儿或新生儿的临床症状。Anti-Ro/SS-A,抗La/SS-B,在较小程度上,抗U1RNP自身抗体(aAbs)与NLE的相关性最强。然而,尽管携带了这些aAbs,但约50%的受影响母亲无症状。该疾病的临床表现非常多样化。心脏表现是最严重的,包括先天性心脏传导阻滞(CHB),死亡率约为18%。羟氯喹(HCQ)的预防性治疗可将随后怀孕的CHB复发率降低约50%。