肺纤维化合并肺气肿综合征

定义

肺纤维化合并肺气肿综合征（CPFE）是一种由上叶肺气肿和下叶肺纤维化组成的综合征，在胸部计算机断层扫描上定义，其特征是肺活量低于正常值，气体交换严重受损，肺动脉高压发病率高，且生存率低。 CPEF的功能特征是肺部一氧化碳扩散能力严重受损，运动时出现低氧血症，但肺容积保持不变。尽管肺活量低于正常值，这可能是导致其识别不足的原因。但CPFE是一个严重的实体。诊断时是否存在肺动脉高压是决定预后的一个关键因素。

Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome of combined emphysema of the upper lobes and fibrosis of the lower lobes defined on chest computed tomography, and characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival. Characteristic functional profile of CPEF is strongly impaired carbon monoxide diffusing capacity of the lung, and hypoxaemia at exercise, with preserved lung volumes. Despite subnormal spirometry, which may be responsible for its under recognition, CPFE is a severe entity. The presence of pulmonary arterial hypertension at diagnosis is a critical determinant of prognosis.

索引词 可用于检索该条目的同义词与别名

• 肺纤维化合并肺气肿综合征
• CPFE[肺纤维化合并肺气肿综合征]