结缔组织病与肺动脉高压

肺动脉高压是多种结缔组织病在呼吸系统的表现之一，它的发病机理尚不完全明确，由于其症状出现晚且不典型，多数患者在确诊时已发展至肺源性心脏病，治疗难度增加，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。本文就结缔组织病相关的肺动脉高压的流行病学、发病机理、治疗等方面作以综述。

Connective Tissue Disease and Pulmonary Hypertension

Pulmonary hypertension is a well-known life threatening complication of several connective tissue diseases. The etiology of the pulmonary hypertension in connective tissue disease remains unknown. Because the prognosis of connective tissue disease associated pulmonary hypertension is generally quite poor, vigilance is required to follow these patients and to identify the development of pulmonary hypertension as early as possible. The occurrence of this complication, pathogenesis, clinical presentation, and treatment options are reviewed in this report.