原发性干燥综合征合并肺动脉高压的临床研究

Clinical Study of Pulmonary Arterial Hypertension in Primary Sjogren's Syndrome

目的探讨原发性干燥综合征（pSS）并发肺动脉高压（PAH）的发生率、临床特点及筛查方法。方法对61例临床确诊为pSS的患者行彩色多普勒超声心动检查，应用三尖瓣反流速度来估测肺动脉收缩压（PASP），依据PAH诊断标准将其分为PAH组（10例）和非PAH组（51例），收集2组的临床资料、实验室检查、胸cT扫描和超声心动的检测结果并进行比较。结果（1）61例pSS患者中并发PAH者占16．4％，PAH组中PASP为31～64mmHg，平均（40±9）mmHg，其中4例无任何与PAH相关的临床表现，PAH的发生与患者年龄、性别、病程无关。（2）患者的主要症状为发热、干咳、胸闷或胸痛、心悸、进行性呼吸困难、雷诺现象、胸腔积液和肺间质病变，PAH组雷诺现象、心悸、肺问质病变的发生率高于非PAH组（P〈0．05），PAH组的补体C3水平低于非PAH组（P〈0．05）。（3）PAH组瓣膜受累达100％，其中2例仅伴有右心系统扩大，3例同时出现左心和右心系统扩大，PAH组左房内径、右房内径、右室内径、肺动脉内径高于非PAH组（P〈0．05或P〈0．01）。结论pSS并发PAH并不少见，患者即使无相应临床症状。也应行超声心动筛查，这对PAH的临床早期诊断和治疗十分重要。

Objective To investigate the incidence, clinical features and screening method of primary Sjogren＇s syndrome （pSS） complicated with pulmonary arterial hypertension （PAH）. Methods Sixty-one patients with clinically diagnosed pSS were detected by color Doppler echocardiography. The pulmonary artery systolic pressure （PASP） was assessed by measuring tricuspid valve reverse flow rate. According to the criteria for the diagnosis of PAH, 61 patients were divided into PAH group （n=10） and non-PAH group （n=51）. The clinical data, laboratory examination, chest CT scan and ultrasound cardiac detection results were collected and compared. Results （1） PAH incidence was 16.4% in 61 pSS patients. Values of PASP were 31 mm Hg to 64 mm Hg in PAH group, and average was （40±9） mm Hg. Four cases of them had no PAH related clinical manifestation. The occurrence of PAH was not related to patient＇s age, gender and course. （2） The main symptoms were fever, dry cough, chest distress or chest pain, heart palpitation, progressive dyspnea, Raynaud＇s phenomenon, pleural effusion, and interstitial pulmonary disease. There was a strong correlation between PAH and Raynaud＇s phenomenon, heart palpitation and interstitial lung disease （P 〈 0.05）. The level of complement C3 was significantly lower in PAH group than that of non-PAH group （P 〈 0.05）. （3） The valve involvement was 100% in PAH group, among them 2 cases with only expanded right heart system, 3 cases with left heart and right heart system expanded. The left atrial inner diameter, right atrial inner diameter, right ventricular inner diameter and pulmonary artery inner diameter were significantly higher in PAH group than those of non-PAH group （P 〈 0.05 or P 〈 0.01）. Conclusion pSS patients with PAH is not rare. Patients even in the absence of clinical symptoms also should be performed echocardiography screening, which is important for early diagnosis and treatment.