Betelli Mauro, Breda Silvia, Ramoni Veronique, Parisi Federico, Rampello Stefania, Limonta Massimiliano, Meroni Marianna, Brucato Antonio
Internal Medicine, Bolognini Hospital, Bergamo - Italy.
Internal Medicine, Papa Giovanni XXIII Hospital, Bergamo - Italy.
J Scleroderma Relat Disord. 2018 Feb;3(1):21-29. doi: 10.1177/2397198317747440. Epub 2018 Apr 4.
This comprehensive review summarizes retrospective and prospective studies on pregnancy in systemic sclerosis in order to educate physicians on critical management issues. Fertility is normal in women with established systemic sclerosis. Their rates of spontaneous losses are comparable to the general population, except for patients with late diffuse systemic sclerosis and severe internal organ involvement who may have higher risks of abortion. Prematurity is clearly higher among systemic sclerosis women, similarly to other rheumatic diseases such as systemic lupus erythematosus and anti-phospholipid antibody syndrome. A placental vasculopathy has been observed in some women with systemic sclerosis. Overall, the disease generally remains stable in most pregnancies. Women with pulmonary hypertension should avoid pregnancy on account of the high maternal mortality risk. Management of systemic sclerosis patients before and during pregnancy includes evaluation of organ involvement and autoantibody analysis, preconceptional folic acid, and discontinuation of drugs with teratogenic potential (bosentan, mycophenolate mofetil, methotrexate, etc.). Management by high-risk pregnancy teams including neonatologists is very important to ensure the best outcomes.
这篇综述总结了系统性硬化症患者妊娠的回顾性和前瞻性研究，以便让医生了解关键的管理问题。已确诊系统性硬化症的女性生育能力正常。她们的自然流产率与普通人群相当，但晚期弥漫性系统性硬化症且伴有严重内脏器官受累的患者流产风险可能更高。与系统性红斑狼疮和抗磷脂抗体综合征等其他风湿性疾病类似，系统性硬化症女性的早产率明显更高。在一些系统性硬化症女性中观察到胎盘血管病变。总体而言，大多数妊娠期间疾病通常保持稳定。患有肺动脉高压的女性因孕产妇死亡风险高应避免妊娠。系统性硬化症患者妊娠前和妊娠期间的管理包括评估器官受累情况和自身抗体分析、孕前补充叶酸以及停用具有致畸潜力的药物（波生坦、霉酚酸酯、甲氨蝶呤等）。由包括新生儿科医生在内的高危妊娠团队进行管理对于确保最佳结局非常重要。
