[Systemic sclerosis and pregnancy]

Pregnancy in women diagnosed with systemic sclerosis is an infrequent situation with a generally favourable outcome according to the most recent studies. Women with systemic sclerosis who wish to become pregnant should obtain preconception counselling in order to withdraw teratogenic drugs and to carefully assess the disease activity. Severe organ impairment, early diffuse systemic sclerosis and pulmonary hypertension ought to discourage patients from pregnancy, as these situations are at high risk of complications for both mother and fetus during pregnancy and puerperium. During pregnancy, the most frequent maternal complication is worsening of gastroesophageal reflux, and the most severe complications are renal crisis and flaring arterial pulmonary hypertension. Angiotensin converting enzyme inhibitors are indicated at any stage of the pregnancy if renal crisis is suspected. Adverse obstetric outcomes are dominated by prematurity which may be the consequence of intra-uterine growth restriction or preeclampsia. Those complications might account for systemic sclerosis-associated vasculopathy, or for the presence of antiphospholipid antibodies. Patients with a previous history of placental insufficiency might benefit from treatment with aspirin, low dose anticoagulants or even nitric oxide donors. During the pregnancy, abnormal bilateral uterine artery Doppler is a predictor of vascular insufficiency and might guide initiation of further preventive treatments. Multicentric prospective cohort studies are warranted to identify more precisely the predictors of pregnancy-related complications and to define the best management of these patients.