一名原发性干燥综合征母亲所生儿童发生晚发型新生儿红斑狼疮。
Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjögren's syndrome.
作者信息
Manthorpe R, Svensson A, Wirestrand L E
机构信息
Sjögren's Syndrome Research Centre in Malmö, Jägersrovägen 80, SE-212 37 Malmö, Sweden.
出版信息
Ann Rheum Dis. 2004 Nov;63(11):1496-7. doi: 10.1136/ard.2003.014944.
BACKGROUND
The neonatal lupus syndrome can be present as congenital heart block (CHB) or as neonatal lupus erythematosus (NLE), both seldom passively acquired autoimmune diseases. CHB starts around week 20 of pregnancy and is a lifelong event, whereas NLE is self limiting and usually starts at the 6th week of the child's age-the maximum recorded up to week 20.
CASE REPORT
An asymptomatic mother with primary Sjogren's syndrome and anti-SSA/Ro52, anti-SSA/Ro60, and anti-SSB/La autoantibodies is described who, at gestational week 23 during her first pregnancy, was diagnosed as having a male fetus with CHB due to third degree atrioventricular block. The boy from the second pregnancy developed skin eruptions which clinically and by biopsy were compatible with NLE at week 20+1 post partum.
CONCLUSIONS
Our case of NLE, starting at week 20+1 of age, seems to be the latest reported clinical case of NLE. Development of CHB and NLE in two consecutive boy pregnancies is unusual.
背景
新生儿狼疮综合征可表现为先天性心脏传导阻滞（CHB）或新生儿红斑狼疮（NLE），这两种均为罕见的被动获得性自身免疫性疾病。CHB在妊娠约20周时开始，是一种终身性疾病，而NLE具有自限性，通常在患儿6周龄时开始——记录到的最大发病年龄为20周。
病例报告
描述了一位无症状的母亲，患有原发性干燥综合征，存在抗SSA/Ro52、抗SSA/Ro60和抗SSB/La自身抗体。在其首次妊娠的第23孕周时，因三度房室传导阻滞被诊断怀有一名患有CHB的男性胎儿。二胎的男孩在产后20 + 1周出现皮肤疹，经临床和活检诊断为与NLE相符。
结论
我们报道的这例NLE病例在20 + 1周龄时发病，似乎是已报道的NLE临床病例中发病年龄最晚的。连续两胎男孩分别发生CHB和NLE的情况较为罕见。
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