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新生儿狼疮所致先天性心脏传导阻滞的预防、筛查和治疗:对医疗服务提供者实践情况的调查。

The prevention, screening and treatment of congenital heart block from neonatal lupus: a survey of provider practices.

机构信息

Division of Rheumatology and Immunology, Duke University Medical Center, Durham, NC.

Department of Pediatrics, Division of Dysmorphology and Teratology, University of California San Diego, San Diego, CA.

出版信息

Rheumatology (Oxford). 2018 Jul 1;57(suppl_5):v9-v17. doi: 10.1093/rheumatology/key141.

Abstract

OBJECTIVE

To survey an international sample of providers to determine their current practices for the prevention, screening, and treatment of congenital heart block (CHB) due to maternal Ro/SSA antibodies.

METHODS

A survey was designed by the organizing committee of the 9th International Conference of Reproduction, Pregnancy and Rheumatic Diseases. It was sent to attendants of the conference and authors of recent publications or abstracts at ACR 2012, 2013 or 2014 on rheumatic diseases and pregnancy.

RESULTS

In anti-Ro/SSA positive women, 80% of 49 respondents recommended screening by serial fetal echocardiogram (ECHO), with most starting at week 16 (59%) and stopping at week 28 (25%), although the time to stop varied widely. For women without a prior infant with neonatal lupus, respondents recommend every other week (44%) or weekly (28%) fetal ECHOs. For women with a prior infant with neonatal lupus, 80% recommend weekly fetal ECHOs. To prevent CHB, HCQ was recommended by 67% of respondents and most would start pre-pregnancy (62%). Respondents were asked about medications to treat varying degrees of CHB in a 20-week pregnant, anti-Ro and La positive SLE patient. For first degree, respondents recommended starting dexamethasone (53%) or HCQ (43%). For second degree, respondents recommended starting dexamethasone (88%). For third degree, respondents recommended starting dexamethasone (55%) or IVIg (33%), although 27% would not start treatment.

CONCLUSION

Despite the absence of official guidelines, many physicians with a focus on pregnancy and rheumatic disease have developed similar patterns in the screening, prevention and treatment of CHB.

摘要

目的

调查国际范围内的医疗服务提供者,以了解他们在预防、筛查和治疗由母体 Ro/SSA 抗体引起的先天性心脏阻滞(CHB)方面的当前实践情况。

方法

由第九届国际生殖、妊娠和风湿疾病会议组织委员会设计了一份调查问卷,分发给会议的与会者以及在 2012、2013 或 2014 年的 ACR 上发表过有关风湿性疾病和妊娠的近期出版物或摘要的作者。

结果

在抗 Ro/SSA 阳性的女性中,49 名受访者中有 80%建议进行连续胎儿超声心动图(ECHO)筛查,大多数从第 16 周(59%)开始,第 28 周(25%)停止,尽管停止的时间差异很大。对于没有新生儿狼疮既往婴儿的女性,受访者建议每两周(44%)或每周(28%)进行胎儿 ECHO。对于有新生儿狼疮既往婴儿的女性,80%建议每周进行胎儿 ECHO。为了预防 CHB,67%的受访者建议使用 HCQ,大多数人会在孕前开始使用(62%)。受访者被问及在一名 20 周妊娠、抗 Ro 和 La 阳性的 SLE 患者中,不同程度 CHB 的治疗药物。对于一级 CHB,受访者建议开始使用地塞米松(53%)或 HCQ(43%)。对于二级 CHB,受访者建议开始使用地塞米松(88%)。对于三级 CHB,受访者建议开始使用地塞米松(55%)或 IVIg(33%),尽管 27%的受访者不会开始治疗。

结论

尽管缺乏官方指南,但许多专注于妊娠和风湿性疾病的医生在 CHB 的筛查、预防和治疗方面已经制定了类似的模式。

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