系统性红斑狼疮并发肺动脉高压的临床回顾性分析

Clinical analysis of patients with systemic lupus erythematosus and concomitant pulmonary hypertension

目的分析系统性红斑狼疮(SLE)合并肺动脉高压(PAH)的临床特点,讨论PAH在SLE中的发生率、致病原因以及PAH的相关临床特征,从而提高对SLE继发PAH的认识。方法回顾性分析2001年1月至2007年12月在我院初诊的798例SLE患者中39例经超声心动图诊断明确、资料完整的合并PAH患者。结果本组SLE患者合并PAH的患病率为4.9%,此39例患者年龄14～77岁,平均(33±12)岁,男性5例,女性34例。其中雷诺现象、指端血管炎、抗u1RNP阳性、抗心磷脂抗体IgG阳性、心包积液、SLEDAI评分高和肺间质病变与出现PAH的发生率成正相关。结论SLE是自身免疫性疾病中合并PAH的较常见的疾病,对于出现雷诺现象、指端血管炎、抗u1RNP阳性、抗心磷脂抗体IgG阳性以及心包积液和肺间质病变的SLE患者应警惕PAH的可能,超声心动图及相关检查有利于早期诊断。

Objective To investigate the clinical manifestations, diagnosis and interventions of pulmonary hypertension （PAH） in patients with systemic lupus erythematosus （SLE）. Methods From January 2001 to December 2007, 798 SLE patients without prior diagnosis were admitted in our hospital, among whom 39 were identified to have concomitant PAH defined by echocardiography. The clinical data of the 39 cases were analyzed retrospectively. Results The incidence of PAH was 4.9% in these cohort of SLE patients. The 39 SLE patients with concomitant PAH included 5 men and 34 women with a mean age of 34±12 years. Positive correlations were found between the occurrence.ofPAH and the Raynaud phenomenon, fingertip vasculitis, anti-ulRNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia （P〈0.05）. Patients with higher scores for SLE Disease Activity Index were liable to PAH. The presence of Raynaud phenomenon, fingertip vaseulitis, anti-ulRNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia is correlated to greater severity PAH with poor prognosis. Conclusions PAH is not a rare concomitant disease in SLE patients. The presence of Raynaud phenomenon, fingertip vasculitis, anti-ulRNP antibody positivity, antiphospholipid antibody positivity, pericardial effusion, and interstitial pneumonia all suggest the likeliness of PAH in SLE patients, and echocardiographic examination may help derive an early diagnosis.