摘要
特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是指原因不明并以普通型间质性肺炎(usual interstitial pneumonia,UIP)为特征性病理改变的一种慢性炎症性间质性疾病,主要表现为弥漫性肺泡炎、肺泡单位结构紊乱和肺纤维化。病情多呈进行性发展,晚期可出现发绀、肺动脉高压(pulmonary arterial hypertension, PAH)、肺源性心脏病、右心功能不全等。
出处
《内科理论与实践》
2008年第6期382-386,共5页
Journal of Internal Medicine Concepts & Practice
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