新生儿狼疮的皮肤表现及后续先天性心脏传导阻滞的风险
Cutaneous manifestations of neonatal lupus and risk of subsequent congenital heart block.
作者信息
Izmirly Peter M, Llanos Carolina, Lee Lela A, Askanase Anca, Kim Mimi Y, Buyon Jill P
机构信息
New York University School of Medicine, New York, New York 10016, USA.
出版信息
Arthritis Rheum. 2010 Apr;62(4):1153-7. doi: 10.1002/art.27333.
OBJECTIVE
Cutaneous disease associated with placental transport of maternal anti-SSA/Ro or anti-SSB/La antibodies is transient, and children often appear to be otherwise healthy. However, the impact of this manifestation of neonatal lupus (NL) on the risk of cardiac disease occurring in a future pregnancy is critical for family counseling and for powering preventive trials. The purpose of this study was to determine the recurrence rates of NL, with specific focus on cardiac NL following cutaneous NL in a child enrolled in the Research Registry for Neonatal Lupus (RRNL).
METHODS
Fifty-eight families who were enrolled in the RRNL met the following inclusion criteria for our study: maternal anti-SSA/Ro or anti-SSB/La antibodies, a child with cutaneous NL, and a pregnancy subsequent to the child with cutaneous NL.
RESULTS
The majority of the 58 mothers (78%) were Caucasian. Of 77 pregnancies that occurred following the birth of a child with cutaneous NL, the overall recurrence rate for any manifestation of NL was 49% (95% confidence interval [95% CI] 37-62%); 14 pregnancies (18.2%) were complicated by cardiac NL, 23 (29.9%) by cutaneous NL, and 1 (1.3%) by hematologic/hepatic NL. A subset analysis was restricted to the 39 children who were born after the initial child with cutaneous NL had been enrolled in the RRNL. The overall recurrence rate for NL was 36% (95% CI 20-52%); 5 pregnancies (12.8%) were complicated by cardiac NL and 9 (23.1%) by cutaneous NL. There were no significant differences in the following maternal risk factors for having a subsequent child with cardiac or cutaneous NL: age, race/ethnicity, anti-SSB/La status, diagnosis, use of nonfluorinated steroids, or breastfeeding. The sex of the subsequent fetus did not influence the development of cardiac or cutaneous NL.
CONCLUSION
Based on data from this large cohort, the identification of cutaneous NL in an anti-SSA/Ro antibody-exposed infant is particularly important, since it predicts a 6-10-fold risk of a subsequent child developing cardiac NL.
目的
与母体抗SSA/Ro或抗SSB/La抗体经胎盘转运相关的皮肤疾病是短暂性的，儿童通常在其他方面看起来健康。然而，新生儿狼疮（NL）的这一表现对未来妊娠中发生心脏病风险的影响，对于家庭咨询和开展预防性试验的力度而言至关重要。本研究的目的是确定NL的复发率，特别关注在新生儿狼疮研究登记处（RRNL）登记的一名儿童出现皮肤型NL后发生心脏型NL的情况。
方法
登记在RRNL的58个家庭符合我们研究的以下纳入标准：母体抗SSA/Ro或抗SSB/La抗体、一名患有皮肤型NL的儿童以及该患有皮肤型NL的儿童之后的一次妊娠。
结果
58名母亲中的大多数（78%）为白种人。在患有皮肤型NL的儿童出生后的77次妊娠中，NL任何表现的总体复发率为49%（95%置信区间[95%CI]37 - 62%）；14次妊娠（18.2%）并发心脏型NL，23次（29.9%）并发皮肤型NL，1次（1.3%）并发血液学/肝脏型NL。一项亚组分析仅限于最初患有皮肤型NL的儿童登记到RRNL之后出生的39名儿童。NL的总体复发率为36%（95%CI 20 - 52%）；5次妊娠（12.8%）并发心脏型NL，9次（23.1%）并发皮肤型NL。在以下母体危险因素方面，即生育后续患有心脏型或皮肤型NL儿童的危险因素方面，不存在显著差异：年龄、种族/族裔、抗SSB/La状态、诊断、非氟化类固醇的使用或母乳喂养。后续胎儿的性别不影响心脏型或皮肤型NL的发生。
结论
基于这个大型队列的数据，在暴露于抗SSA/Ro抗体的婴儿中识别出皮肤型NL尤为重要，因为它预示着后续儿童发生心脏型NL的风险会增加6至10倍。
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