Obstetrical Success of a Very High-Risk Pregnancy in a Patient with Severe Systemic Sclerosis and a History of Three Fetal Deaths and One Neonatal Death

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This case report describes the successful multidisciplinary management, within a specialized facility, of a very high-risk pregnancy in a 42-year-old patient with systemic sclerosis (SSc). The disease was stable under azathioprine (150 mg/day) and characterized by double immunological positivity (ANA, anti-centromere) along with severe dual involvement: mucocutaneous and interstitial lung disease (pure restrictive ventilatory defect with TLC < 51%). Despite a tragic obstetric history marked by three fetal deaths and one early neonatal death, intensive bi-weekly maternal-fetal monitoring involving obstetricians, internists, cardiologists, and pulmonologists maintained maternal systemic stability (with no scleroderma renal crisis or pulmonary arterial hypertension) and ensured harmonious fetal growth (eutrophic fetus). At the end of the third trimester, given the major risk of maternal respiratory exhaustion during pushing efforts and the strict contraindications to general anesthesia (risks of difficult intubation due to oral sclerosis, aspiration pneumonitis due to severe gastroesophageal reflux, and acute PAH flare-up during induction), a prophylactic cesarean section under spinal anesthesia was planned. The spontaneous onset of labor at 36 weeks and 6 days prompted an emergency performance of the procedure, leading to the birth of a healthy female infant weighing 2950 g with an Apgar score of 10/10 and uneventful postoperative recovery.

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