结缔组织病相关的肺动脉高压79例临床分析

目的 了解结缔组织病相关的肺动脉高压(PAH)的发生率,评估临床特点与PAH发生的相关性、探讨早期诊断及治疗方法.方法 从1892例结缔组织病患者中筛选出伴有肺动脉高压的患者79例,对其临床实验室检查特点及治疗转归进行回顾性分析,找出并发PAH相关的危险因素,评价不同治疗、不同原发病对预后的影响.结果 结缔组织病合并的PAH的总发生率约为4.2%.其中系统性硬化症(SSc)合并肺动脉高压发生率最高,为18.18%.混合性结缔组织病(MCTD)次之12.00%,显著高于皮肌炎/多发性肌炎、系统性红斑狼疮、干燥综合征、类风湿关节炎、抗磷脂综合征等结缔组织病(P＜0.01).临床伴有雷诺现象者肺动脉压力显著高于无雷诺现象者(P＜0.01).高球蛋白血症、抗U1RNP(+)、ACA-IgG(+)、心包积液、肺问质病变的结缔组织病患者更易出现PAH,且与严重程度呈相关性(P＜0.05).结论 结缔组织病中PAH是较为常见的并发症,其中在SSc和MCTD发生率最高.雷诺现象、抗U1RNP阳性、ACA-IgG(+)提示结缔组织病患者有更易发生PAH的可能.

Clinical analysis of 79 pulmonary arterial hypertension cases from 1892 connective tissue disease patients

Objective To understand the prevalence, investigate the correlation of clinical features, explore the early-stage diagnosis and treatment of pulmonary arterial hypertension(PAH)in patients with connective tissue disease(CTD).Methods All cases with pulmonary arterial hypertension in 1892 CTD patients were analyzed retrospectively. The risk factor of PAH was evaluated and the prognostic influence of difierent treatments and primary diseases analyzed. Results The prevalence of PAH in patients with connective tissue disease wag about 4.2%(79/1 892).In these patients, systemic sclerosis (SSc)and mixed connective tissue disease(MCTD)had the highest incidence of PAH(18.18%and 12.00%)(P＜0.01).It was obviously higher than polymyositis/dermatomyositis(6.2%),systemic lupus erythematosus(4.4%),Sjogren syndrome(3.8%),rheumatoid arthritis(0.8%)and anti-phospholipid syndrome(0.5%),etc.(P＜0.01).Raynaud's phenomenon was related to a higher pulmonary arterial pressure(P＜0.01).There was a positive correlation(P＜0.01)between the presence of Raynaud's phenomenon and pulmonary arterial pressure. Abnormal lung function was a common finding. There were associations(P＜0.05)between the degree of pulmonary hypertension and IgG,anti-U1RNP antibody positive, antiphospholipid antibody positive, pericardial effusion and interstitial pneumonia. Conclusion PAH is common in connective tissue disease. SSc and MCTD have the highest prevalence of PAH. The presence of Raynaud's phenomenon anti-U1RNP antibody is positively correlated with pulmonary arterial pressure. It can predict the development of PAH. It is useful to perform ultraechocardiogrphy for an earlystage diagnosis and prognostic analysis.