原发性干燥综合征患者肺动脉压力变化的临床研究

Clinical study on pulmonary arterial pressure in interstitial lung disease patients with primary Sjogren's syndrome

目的探讨合并间质性肺病（ILD）的原发性干燥综合征（pSS）患者肺动脉压力变化及其与肺功能、预后的关系。方法分析南京市鼓楼医院及南京鼓楼医院集团宿迁市人民医院2009年1月至2013年1月确诊的pSS-ILD患者108例,按超声心动图检测的肺动脉收缩压（SPAP）结果,依据肺动脉高压（PAH）诊断标准将其分为PAH组（26例）和非PAH组（82例）,收集两组的临床资料、超声心动图、肺功能的检测结果,电话随访患者的用药、治疗及死亡情况,并行PAH组的肺动脉收缩压与肺功能指标相关性研究。结果（1）108例pSS-ILD患者中有PAH者26例（24.0%）,PAH组中SPAP为35～82 mmHg,平均（56.8±9.2）mmHg,PAH的发生与患者年龄、性别、病程无关。（2）PAH组FVC%、TLC%、DLCO%较非PAH组降低（P〈0.05）,肺动脉收缩压与FVC%、TLC%、DLCO%呈负相关,与FEV1/FVC无相关性。随访12月后PAH组再入院率、死亡率较非PAH组升高。结论 pSS-ILD患者合并肺动脉高压后,肺功能减退明显,预后较差。定期对患者行超声心动图和肺功能检查对诊断PAH以进行早期治疗非常必要。

Objective To investigate the change of pulmonary arterial pressure in interstitial lung disease（ ILD） patients with primary Sjogren＇s syndrome（ pSS） and its correlation to lung function and prognosis. Methods From January 2009 to January 2013,108 patients with pSS-ILD were detected by color Doppler echocardiography.According to the criteria for the diagnosis of PAH,108 patients were divided into the PAH group（ n = 26） and the non-PAH group（ n = 82）. The clinical data,lung function and ultrsonic cardiogram detection results were collected,and the correlation between pulmonary artery systolic pressure and lung function was analyzed in the PAH group.Their baseline data and therapies were recorded at one year follow-up. Results（ 1） The incidence of PAH was24. 0% in 108 pSS-ILD patients. The values of SPAP were 35 mmHg to 82 mmHg in the PAH group,and the average was（ 56. 8 ± 9. 2） mmHg. The occurrence of PAH was not related to patient＇s age,gender and course.（ 2） The PAH group had lower FVC% pred,TLC% pred and DLCO% pred compared with those of the non-PAH group. In the PAH group,SPAP had positive correlation with FVC% pred,TLC% pred,DLCO% pred and no correlation with FEVl /FVC. A significantly higher incidence of re-hospitalization and mortality was observed in the PAH group. Conclusion In the PAH group,their lung function declines significantly,PAH is an important factor related to the prognosis of pSS-ILD,people should be performed lung function and ultrasonic echocardiogram after the diagnosis,which is important for early diagnosis and treatment.