系统性硬皮病相关肺动脉高压  

Pulmonary arterial hypertension associated with systemic seleroderma

作　　者：李小静[1] 张磊[1] 孙建方[1] 

机构地区：[1]中国医学科学院北京协和医学院皮肤病研究所,南京210042

出　　处：《国际皮肤性病学杂志》2009年第5期300-302,共3页International Journal of Dermatology and Venereology

摘　　要：肺部是系统性硬皮病最常累及的脏器之一。作为硬皮病患者死亡的主要原因，肺动脉高压可以是系统性硬皮病的一种原发性临床表现，也可以继发于间质性肺纤维化。系统性硬皮病相关肺动脉高压早期可无任何症状或症状不明显，诊断较困难，但肺功能测定可发现气体弥散功能障碍。因此，认识系统性硬皮病相关肺动脉高压的危险因素及病理生理过程，可早期识别、诊断及进一步优化治疗方案。Lung is a most commonly involved organ in systemic scleroderma （SS）. As a predominate reason for the death of patients with SS, pulmonary hypertension may be a primary manifestation of SS or a secondary manifestation following interstitial pulmonary fibrosis. It is difficult to diagnose pulmonary hypertension at early stage since symptoms may be absent or just unobvious in patients, while pulmonary function test, which can reveal gas diffusion dysfunction at early stage, may benefit the early diagnosis of it. To understand the risk factors and pathogenesis of SS-associated pulmonary hypertension may facilitate the early recognition, diagnosis, and optimization of treatment of this entity.

关 键 词：硬皮病 系统性 高血压 肺性 治疗 

分 类 号：R593.25[医药卫生—内科学] R543.2[医药卫生—临床医学]