Jersey City Medical Center, Rutgers New Jersey Medical School, Newark, NJ, USA.
J Matern Fetal Neonatal Med. 2024 Dec;37(1):2411583. doi: 10.1080/14767058.2024.2411583. Epub 2024 Oct 3.
Women with Sjögren's Disease are more likely to experience pregnancy complications compared to their counterparts without the disease. Attention to detail and familiarity with the most recent research and guidelines in this field are required to achieve optimal maternal and fetal outcomes. Such complications include pregnancy induced hypertension, fetal growth restriction, thromboembolic events, and preterm delivery. Among the most life-threatening sequela of maternal Sjogren's Disease is fetal autoimmune congenital heart block (ACHB), which has high potential to cause intrauterine fetal death, neonatal mortality, developmental delay, and other long-term pediatric complications. Currently, surveillance with weekly echocardiograms and obstetric sonograms in the second trimester are recommended to screen for ACHB with the goal of early detection and intervention before progression from first- or second- of heart block to complete heart block.
We describe a case of maternal Sjogren's Disease, which prompted us to raise questions regarding the optimal frequency of obtaining fetal echocardiograms, and the ideal management in case a prolonged PR interval was to be found. We use this case to provide a springboard for discussion on updated antenatal management strategies for ACHB prevention.
To conduct this analysis, we searched PubMed for articles published over the last 10 years, with attention focused on articles written since 2016. Additionally, updated guidelines by other specialties such as Rheumatology, Cardiology and Pediatrics on this issue were reviewed.
Thorough search of the literature yielded several meta-analyses concurring that the mothers with Sjogren's Disease had increased rates of premature birth, pregnancy induced hypertension, increased risks of delivering infants with intrauterine growth restriction (IUGR), with the most life-threatening risk being that of congenital heart block. Literature supporting prophylactic hydroxychloroquine and the use of steroids to reverse or halt the progression of congenital heart block at the time of diagnoses appeared at the forefront of search results.
Pregnant women with SS have an increased risk for complications such as intrauterine growth restriction, thromboembolic events, pregnancy-induced hypertension, preterm delivery, and cesarean delivery and should prioritize obtaining pre- or peri-conceptional counseling. In women with anti SSA/SSB antibodies, a medication regimen should be considered with the object of decreasing the concentration of these antibodies, and hence decrease the risks of ACHB. Current literature supports the inclusion of hydroxychloroquine for this purpose, even prior to conception. Although the most recent studies recommend against prophylactic use of steroids, their potential to prevent progression to complete block should be weighed against their potential negative effects. Short and long-term treatment with corticosteroids has been associated with increased maternal risk of infection, weight gain, osteonecrosis, hypertension and bone mineral density disorders. Intrauterine growth restriction, oligohydramnios, and adrenal suppression have been among the fetal risks associated with steroids while improved infant survival or decreased need for pacing have not been demonstrated. Management of these pregnancies is complex and should include a multidisciplinary approach involving a maternal-fetal medicine sub-specialist, a rheumatologist, a pediatrician, a neonatologist, and the patient herself with her family in a model of shared decision-making.
与没有这种疾病的女性相比，患有干燥综合征的女性更有可能经历妊娠并发症。为了实现母婴的最佳结局，需要注意细节，熟悉该领域的最新研究和指南。这些并发症包括妊娠高血压、胎儿生长受限、血栓栓塞事件和早产。在母体干燥综合征最具威胁生命的后果中，有一种是胎儿自身免疫性先天性心脏传导阻滞（ACHB），它有很高的潜在风险导致宫内胎儿死亡、新生儿死亡率、发育迟缓以及其他长期儿科并发症。目前，建议在妊娠中期进行每周一次的超声心动图和产科超声检查，以筛查 ACHB，目的是在第一或第二度心脏传导阻滞进展为完全性心脏传导阻滞之前进行早期发现和干预。
我们描述了一例母体干燥综合征的病例，这促使我们提出了关于获得胎儿超声心动图的最佳频率以及发现延长 PR 间隔时的理想管理的问题。我们利用这个病例为讨论更新 ACHB 预防的产前管理策略提供了一个跳板。
为了进行这项分析，我们在过去 10 年中在 PubMed 上搜索了文章，并特别关注自 2016 年以来发表的文章。此外，还审查了风湿病学、心脏病学和儿科学等其他专业领域关于这个问题的最新指南。
对文献的彻底搜索得出了几项荟萃分析的结论，即患有干燥综合征的母亲早产、妊娠高血压、胎儿宫内生长受限（IUGR）的风险增加，最具威胁生命的风险是先天性心脏传导阻滞。在诊断时使用羟氯喹预防和使用类固醇逆转或阻止先天性心脏传导阻滞进展的文献出现在搜索结果的最前沿。
患有 SS 的孕妇发生并发症的风险增加，例如宫内生长受限、血栓栓塞事件、妊娠高血压、早产和剖宫产，应优先进行预产前或围产期咨询。对于抗 SSA/SSB 抗体阳性的女性，应考虑采用药物治疗方案，以降低这些抗体的浓度，从而降低 ACHB 的风险。目前的文献支持为此目的使用羟氯喹，甚至在怀孕之前。尽管最近的研究不建议预防性使用类固醇，但它们预防进展为完全性阻滞的潜力应该与它们的潜在副作用相权衡。短期和长期使用皮质类固醇与母亲感染、体重增加、骨坏死、高血压和骨矿物质密度紊乱的风险增加有关。与类固醇相关的胎儿风险包括宫内生长受限、羊水过少和肾上腺抑制，而婴儿存活率提高或起搏需求减少尚未得到证实。这些妊娠的管理很复杂，应包括多学科方法，涉及母体胎儿医学专家、风湿病学家、儿科医生、新生儿科医生以及患者及其家属的共同决策模式。
