Pregnancy in women with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) remains a high risk. We successfully managed a pregnancy in a patient with SLE-PAH. A 31-year-old pregnant woman with SLE-PAH had worsening PAH and SLE flare-up during pregnancy and a sudden increase in pulmonary arterial pressure after delivery. SLE-PAH was controlled by continuous intravenous epoprostenol and inhaled nitric oxide therapy combined with high-dose corticosteroids under close hemodynamic monitoring. Women with SLE-PAH should avoid pregnancy. However, in case of a similar event, we recommend our case as a good reference for improving the outcome of pregnancy with SLE-PAH.
Keywords: pregnancy; pulmonary arterial hypertension; systemic lupus erythematosus.