系统性红斑狼疮合并肺动脉高压患者临床特点分析

Systemic lupus erythematosus associated pulmonary arterial hypertension： clinical analysis of 91 cases

目的 分析系统性红斑狼疮（SLE）合并肺动脉高压（PAH）患者的临床特点和疗效.方法 对上海交通大学医学院附属仁济医院2007至201 1年91例患者的临床资料进行分析并作随访.根据首诊时患者NYHA（纽约心脏协会）心功能评级分为两组,A组：心功能1～2级,B组：心功能3～4级.结果 91例患者男女比例为2∶89,年龄（37±11）岁,SLE病程（7±6）年.10例患者PAH是SLE的首发表现.心脏彩超（UCG）估测的肺动脉收缩压（PASP）为40～128 mmHg.行右心导管（RHC）检查18例,占19.78％,导管实测PASP与UCG估测值对应性良好.91例中较多发生雷诺症49例（53.8％）、心包积液47例（51.6％）和抗RNP抗体阳性52例（57.14％）.轻中度患者PASP与SLE疾病活动有正相关.观察期内死亡27例（29.67％）;其中A组4例（14.8％）,B组23例（85.2％）.A组死因多为非心源性,B组多死于右心功能衰竭.环磷酰胺对PASP＜ 70 mmHg患者有一定效果.44例患者应用了各类PAH靶向药物,有显著疗效,显著延长了重症患者的生存期.结论 SLE-PAH患者主要的临床特点包括雷诺氏症、心包积液和抗RNP抗体阳性.轻中度患者PASP与SLE活动呈现正相关,环磷酰胺静脉冲击治疗能取得一定疗效,重症患者应用PAH靶向药物能显著改善预后.

Objective To explore the clinical characteristics and therapeutic efficacy of systemic lupus erythematosus （SLE） associated pulmonary arterial hypertension （PAH）.Methods A total of 91 cases of SLE-PAH from 2007 to 2011 were reviewed and followed up.They were divided into 2 groups：group A：New York Heart Association （NYHA） functional class 1 and 2; group B：NYHA functional class 3 and 4.Results There were 2 males and 89 females with a mean age of 37 ± 11 years.The mean duration of SLE disease process was 7 ± 6 years.PAH was the primary symptom of SLE onset in 10 cases.Pulmonary arterial systolic pressure （PASP） as measured by ultrasonic cardiography （UCG） were between 40 to 128 mmHg.Eighteen cases （19.78％） underwent right heart catheterization （RHC）.There were good parallels of PASP value between RHC and UCG.The main characteristics included Raynaud＇ s phenomenon （53.8％）,pericardial effusion （51.6％） and a high titer of anti-RNP antibody （57.1％）.PASP was positively associated with SLE disease activity in mild and moderate cases.Among 27 mortality cases,there were 4 in group A（14.8％）and 23 in group B（85.2％）.And the causes of immortality were mostly noncardiac in group A and right heart failure in group B.Cyclophosphamide was effective in mild and moderate cases.Forty-four cases received PAH target treatment and it could decrease the PASP in mild and moderate cases and significantly prolong the survival for severe cases.Conclusion The major clinical characteristics of SLE-PAH patients include Raynaud＇s phenomenon,pericardial effusion and positivity of anti-RNP antibody.PASP is positively associated with SLE disease activity in mild and moderate cases for whom intravenous pulse cyclophosphamide therapy may be effective.For severe cases,concomitant PAH target therapy may significantly improve the prognosis.