Objective

Studies evaluating pregnancy outcomes in systemic sclerosis (SSc) are limited. SSc is associated with maternal complications and adverse neonatal outcomes. This study investigated the impact of disease stage (stable vs active) on the maternal and neonatal outcomes of pregnancies of patients followed at an Israeli medical centre.

Method

The charts of 354 SSc female patients followed during 2003–2020 were reviewed. Data on clinical and laboratory features, number of pregnancies close to SSc diagnosis, and maternal and neonatal outcomes were analysed. Patients were divided into a stable disease and an active/early disease group.

Results

The active/early disease group included 26 patients [19 diffuse SSc (dSSc)], with 38 pregnancies. Median disease duration was 1 year, except for four patients who were first diagnosed during pregnancy. SSc was exacerbated in all patients during pregnancy or shortly after delivery [skin, lung, and heart involvement in dSSc; severe vasculopathy in limited SSc patients]. Six patients had hypertensive disorders of pregnancy; four pregnancies ended with foetal death. Thirty-three children were born, 60% with intrauterine growth retardation (IUGR)/low birthweight (LBW). The stable disease group included 19 patients, including seven with previously active disease, now stabilized (five dSSc), and 32 pregnancies. All pregnancies were planned and monitored closely. Disease remained stable in all patients. Four patients had hypertensive disorders of pregnancy; 12/29 newborns had LBW (41%).

Conclusion

Active maternal disease during pregnancy poses an increased risk of SSc aggravation. The maternal and neonatal outcomes in planned pregnancy during stable disease are favourable. IUGR/LBW is common among neonates, even in stable disease.

中文翻译：

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系统性硬化症患者在疾病稳定期间计划怀孕的有利结果

客观的

评估系统性硬化症 (SSc) 妊娠结局的研究有限。SSc 与产妇并发症和不良新生儿结局有关。本研究调查了疾病分期（稳定与活跃）对以色列医疗中心随访患者的孕产妇和新生儿结局的影响。

方法

审查了 2003 年至 2020 年间随访的 354 名 SSc 女性患者的图表。分析了有关临床和实验室特征、接近 SSc 诊断的妊娠数以及孕产妇和新生儿结局的数据。患者被分为疾病稳定组和活动性/早期疾病组。

结果

活动性/早期疾病组包括 26 名患者 [19 名弥漫性 SSc (dSSc)]，其中 38 名患者怀孕。中位病程为 1 年，除了 4 名在怀孕期间首次诊断的患者。所有患者在怀孕期间或分娩后不久都会加重 SSc [dSSc 中的皮肤、肺和心脏受累；局限性 SSc 患者的严重血管病变]。六名患者患有妊娠期高血压疾病；四次怀孕以胎儿死亡告终。33 个孩子出生，其中 60% 患有宫内发育迟缓 (IUGR)/低出生体重 (LBW)。疾病稳定组包括 19 名患者，其中 7 名先前患有活动性疾病，现在稳定（5 名 dSSc），并且有 32 名患者怀孕。所有怀孕都经过计划和密切监测。所有患者的疾病都保持稳定。4名患者患有妊娠期高血压疾病；12/29 的新生儿有 LBW (41%)。

结论

怀孕期间活跃的母体疾病会增加 SSc 恶化的风险。在疾病稳定期间计划妊娠的母婴结局良好。IUGR/LBW 在新生儿中很常见，即使在病情稳定的情况下也是如此。