结缔组织病相关的肺动脉高压研究进展

肺动脉高压是结缔组织病（CTD）的严重并发症之一。结缔组织病中最常受累的是系统性硬化症（SSc），而混合性结缔组织病（MCTD）、系统性红斑狼疮（SLE）及炎性肌病包括多肌炎和皮肌炎也常被累及。结缔组织病相关的肺动脉高压引起的原因不仅是肺动脉的病变，慢性血栓的形成，自身免疫反应和肺间质病变也是引起肺动脉高压的原因之一。由于其症状出现晚且不典型，预后甚差，死亡率高，因此对结缔组织病相关的肺动脉高压早期诊断，及时合理的治疗十分重要。

Pulmonary arterial hypertension in connective tissue diseases

Pulmonary arterial hypertension is one of the severe complications of connective tissue diseases (CTD). Systemic sclerosis (SSc) is the most common CTD that can develop pulmonary arterial hypertension, while mixed CTD ( MCTD), systemic lupus erythematosus (SLE) and inflammatory myopathy such as polymyositis and dermatomyositis are also likely to develop pulmonary arterial hypertension. The causes of the CTD associated pulmonary arterial hypertension include not only the changes of the pulmonary artery itself, but also the chronic thromboembolism of the pulmonary artery, auto-immunological reaction and the interstitial pneumonitis. Because of the poor prognosis of the CTD associated pulmonary arterial hypertension, early diagnosis and treatment is very important.