系统性红斑狼疮患者孕期并发肺动脉高压1例

系统性红斑狼疮是一种自身免疫介导的、以免疫性炎症为突出表现的弥漫性结缔组织病，以血清中出现多种自身抗体和多系统如皮肤、关节、肾脏、神经系统、心脏、肺部等受累为临床特征。据统计，我国系统性红斑狼疮的患病率为70/10万［1］，在育龄女性中发病率高达1/1 000［2］。 系统性红斑狼疮患者合并肺动脉高压的发生率为1.8%～14.0%，远低于狼疮性肾炎(47.4%)、系统性红斑狼疮关节受累(54.5%)、系统性红斑狼疮血液系统受累(56.1%)， 甚至低于神经精神性狼疮(4.8%)［3］，但肺动脉高压和疾病的不良预后密切相关［4］。合并肺动脉高压的患者妊娠风险极高，其围产期死亡率高达98/1 000，是造成妊娠合并系统性红斑狼疮患者死亡的首要内科合并症［5］，而多数患者为孕期首次发现，临床处理相对棘手。本文就北京大学第三医院1例系统性红斑狼疮患者孕期并发肺动脉高压分娩的情况报道如下。

A case of systemic lupus erythematosus in pregnancy complicated by pulmonary hypertension

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.