ABSTRACT
Pulmonary hypertension is a rare and potentially life-threatening complication of Systemic lupus erythematosus (SLE), and 5 cases has been previously documented in pregnancy. Four cases died after delivery and only one case was alive. We describe the case of a 28-year-old pregnant woman with pulmonary hypertension related to SLE with no previous history of immunologic disease including SLE. Diagnosis was made at 22 weeks of gestation. Medication including prednisolone and hydroxychloroquinone was commenced immediately and continued throughout the pregnancy. On fetal sonogram, the fetal growth was 3~10 percentile and diastolic notch of uterine arteries was noted. However, a healthy baby girl weighing 2,400 g was born in planned vaginal delivery at gestation week 38. There were no postpartum complications.