系统性红斑狼疮合并肺动脉高压十年住院病例分析

Systemic lupus erythematosus associated pulmonary arterial hypertension： results from lO-year inpa- tient cases analysis in a single center

目的研究系统性红斑狼疮（SEE）合并肺动脉高压患者的临床特点和诊治现状。方法对仁济医院风湿病科2001--2010年间住院患者进行分析随访。根据首诊心脏彩色多普勒超声估测肺动脉收缩压分为A组[≤70mmHg（1mmHg=0．133kPa）]和B组（〉70mmHg）。用Pearson相关分析研究狼疮活动性与肺动脉高压的关系，以X^2检验及Wileoxon检验不同组别的生存曲线差异。结果共有155例患者184次入院记录，占同期狼疮住院患者的4．16％。患者主要特点有：雷诺现象（47．3％）、心包积液（41．9％）和抗RNP抗体阳性（55．4％）。随访例数为132例，47例（35．6％）患者死亡，A组9例（19．1％），B组38例（80．9％）。A组患者肺动脉收缩压与狼疮活动指数呈正相关。单因素分析显示环磷酰胺（P〈0．05）和肺动脉高压靶向治疗药（P〈0．01）与疗效相关。以Logistic回归分析则仅有靶向治疗药与疗效相关（P〈0．01）。应用肺高压靶向药物能显著提高重症患者的1年期生存率。结论本病主要临床特点包括雷诺现象、心包积液和抗RNP抗体阳性。重症患者与狼疮活动性无相关性。肺动脉高压靶向药物疗效确切，环磷酰胺治疗对部分患者有效。重症患者联用环磷酰胺与肺动脉高压靶向药能显著改善预后。

Objective To investigate the clinical characteristics and current condition of treatment for systemic lupus erythematosus （SLE） associated pulmonary arterial hypertension （PAH）. Methods 10-year inpatients cases were reviewed and fallowed up. Cases were divided into 2 groups： group A： patients with baseline pulmonary arterial systolic pressure （PASP） lower than 70 mmHg; group B： patients with baseline PASP higher than 70 mm Hg. Pearson＇s correlation analysis, Chi-square test, Logistic regression, Cox-Mantel and Wilcoxon test were used for statistical analysis. Results There were totally 155 cases with 184 records of admission which accounted for 4.16% among total lupus cases. The main clinical characteristics included Raynaud＇s phenomenon （47.3%）, pericardial effusion （41.9%） and high titer of anti-RNP antibody （55.4%）. There were 132 cases enrolled for prognostic statistical analysis. There were 47 cases of death （35.6%） in total, among which 9 cases （19.1%） were in group A and 38 cases （80.9%） were in group B. In group A, there was a positive correlation between PASP and lupus disease activity index score. Single therapy analysis by Chi-square test showed that cyclophosphamide （CTX） （P〈0.05） and PAH targeted drugs （P〈0.01） were significantly associated with favorable outcome but logistic regressive analysis only confirmed the efficacy of target drugs （P〈0.01）. PAH target drugs significantly improved the one year survival rate of the severe cases. Conclusion The main clinical characteristics of SLE associated PAH include Raynaud＇s phenomenon, pericardial effusion and positive anti-RNP antibody. The severity of PAH may not be related to lupus disease activity. PAH targeted drugs are effective in SLE-PAH. CTX may be effective in some cases. For severe cases, the combination therapy of CTX and PAH targeted drugs could significantly improve the prognosis.