【摘要】 目的分析自身免疫性疾病(AID)合并肺动脉高压的临床特点、诊断以及治疗方法,提高对继发性肺动脉高压的认识。方法对83例诊断明确、资料完整的AID合并肺动脉高压患者进行回顾性分析。结果5年间收治的2016例AID患者中合并肺动脉高压83例(4.1%),年龄10～67岁,平均(33±13)岁,其中男性7例(8.4%),女性76例(91.6%),占同期入院收治的肺动脉高压患者(350例)的31.1%。本组病例中易合并肺动脉高压的AID包括混合性结缔组织病(MCTD)、系统性硬化症(SSc),合并概率最低的为皮肌炎/多发性肌炎(DM/PM)。合并抗磷脂抗体综合征(APS)的系统性红斑狼疮(SLE)患者出现肺动脉高压的概率增加。合并肺动脉高压的AID患者出现雷诺现象的比例很高(P=0),二者存在相关性。出现雷诺现象的患者多有弥散性肺通气障碍,而且病情容易恶化。抗u1RNP阳性的AID患者出现肺动脉高压的可能明显升高(P=0)。结论肺动脉高压是一组以肺小动脉受累的疾病,AID是其常见合并疾病,其中MCTD、SSc合并肺动脉高压的概率最高,DM/PM合并的概率最低。合并APS可以增加SLE出现肺动脉高压的概率。对于出现雷诺现象、抗u1RNP的AID患者应警惕肺动脉高压的可能,早期行超声心动图及相关检查,早期诊断,以改善患者的预后。更多还原

【Abstract】 Objective To investigate the clinical feature, diagnosis and treatment of pulmonary hypertension in autoimmune diseases (AID). Methods All the patients with Doppler-defined pulmonary hypertension among 2016 AID admitted from Jan 1999 to Dec 2004 were analyzed retrospectively. Results Doppler-defined pulmonary hypertension occurred in 350 patients, and 83 cases (4.1% of 2016 patients with AID, 7 men and 76 women; mean±SD age, 33±13) of them were associated with AID. Pulmonary hypertension had a highest incidence in mixed connective tissue disease and progressive systemic sclerosis (P=0), and lowest in dermatomyositis/polymyositis. Antiphospholipid antibody syndrome promoted pulmonary hypertension in systemic lupus erythematosus. There were associations (P=0) between the occurrence of pulmonary hypertension and Raynaud′s phenomenon and anti-u1RNP antibody. Patients with pulmonary hypertension associated with Raynaud′s phenomenon in AID were prone to have reduced carbon monoxide diffusing capacity and were generally severe. Conclusion Pulmonary hypertension, diagnosed by Doppler echocardiography is common in patients with AID, especially in mixed connective tissue disease and progressive systemic sclerosis, but not in dermatomyositis/polymyositis. Antiphospholipid antibody syndrome is a promoting factor for pulmonary hypertension in systemic lupus erythematosus. Raynaud′s phenomenon and anti-u1RNP antibody appear to correlate with pulmonary hypertension in AID.更多还原