干燥综合征合并肺动脉高压6例分析

目的探讨原发性干燥综合征（pSS）中肺动脉高压（PAH）的发生率、临床特点及预后。方法回顾性分析我院自1997年10月至2009年4月收治的pSS患者160例中合并肺动脉高压的6例临床资料。结果所有的患者均有活动后胸憋,均有少量心包积液,67%的患者ANA（＋）抗SSA（-）,伴随肺间质纤维化,33%的患者出现血细胞受累及肾脏受累均为女性,33%有r球蛋白增高均为男性。结论 pSS出现与ANA（＋）,抗SSA（-）,肺间质纤维化有关,临床上出现活动后胸闷的患者应及时检测肺动脉压,尤其是男性患者。肺动脉高压的程度与预后成正相关。

Analysis of 6 patients with Sjogren's syndrome and pulmonary hypertension

Objective To explore the prevalence, clinical features and the treatment of Sjogren＇s syndrome （SS） complicated with pulmonary hypertension. Methods The data of 160 cases in my department with the diagnosis of SS during 1997 to April 2009 were collected and analyzed retrospectively. Six patients complicated with pulmonary hypertension. Results All six patients had exertional dyspnea. All of them had pericardial effusion. 67% patients had positive ANA and 33% female patients had kidney injury c. the elevated gamma-protein were detected in 33% male patients. Conclusion The occurrence of pulmonary hypertension in SS patients-IP is associated with negative SSA and interstitial fibrosis. Pulmonary artery pressure should be measured in order to make early diagnosis. The severity of pulmonary artery hypertension is associated with poor prognosis.