Abstract: To investigate the pathophysiology, screening, diagnosis and treatment of the systemic lupus erythematosus (SLE) in pregnancy complicated with pulmonary hypertension. Retrospective analysis was made of one case of SLE in pregnancy complicated with pulmonary hypertension in Peking University Third Hospital. Literature was reviewed to investigate the pathophysiology, screening, diagnosis and treatment of the SLE in pregnancy complicated with pulmonary hypertension. SLE is an autoimmune mediated diffuse connective tissue disease characterized by immunological inflammation. The incidence of SLE combined with pulmonary hypertension was low, but the risk was high with pregnancy, with a high perinatal mortality rate. Pulmonary hypertension is the leading cause of the death in patients with pregnancy complicated with SLE. This patient was diagnosed with SLE six years before and was hospitalized for 29 weeks of menopause, with fatigue five months and chest congestion one month. Ultrasonic cardiogram showed severe pulmonary hypertension, with pulmonary arterial systolic pressure being 96 mmHg. After admission, multidisciplinary consultation was organized to draw up a diagnosis and treatment plan. The patient received the treatment of rest，oxygen，blood gas and pulmonary artery pressure monitoring. Considering perioperative pulmonary hypertension crisis likely to occur，pulmonary artery catheter was placed preoperatively. In general anesthesia，the cesarean was performed．After operation，the patient was transferred to the intensive care unit, with the treatment of expanding blood vessels，reducing pulmonary artery pressure，administering anticoagulation and preventing infection. Ten days after operation, the patient was discharged from hospital with smooth condition. Strengthening the management of SLE patients in pregnancy, early detection, and cooperation of multidisciplinary teams can help improve maternal and fetal outcomes. Termination of pregnancy should be offered to the patients with severe pulmonary hypertension. Right cardiac catheterization is the gold standard for diagnosing pulmonary hypertension. Some patients are diagnosed in later stage of pregnancy, regular antenatal examination, interdisciplinary co-operation, assessment of cardiac function, monitoring the condition of the pregnant woman and fetus and timely termination of pregnancy are needed. Epidural anesthesia is the appropriate choice for cesarean delivery.

Key words: Systemic lupus erythematosus, Pulmonary arterial hypertension, Pregnancy