肺纤维化合并肺气肿综合征的研究进展

Research progresses of combined pulmonary fibrosis and emphysema syndrome

肺纤维化合并肺气肿（CPFE）综合征近年来逐渐被大家认识和重视。其特征为上肺野为主的肺气肿和下肺野为主的纤维化同时存在；肺功能表现为肺容积相对正常或者轻度异常，而弥散能力明显下降及活动后低氧血症。目前认为这是一类独立的疾病，发病机制尚不明确。CPFE患者常并发肺动脉高压、肺癌和急性肺损伤，预后较单纯肺气肿或肺纤维化差，目前缺少有效治疗手段。本文综述近年来CPFE的研究进展。

Combined pulmonary fibrosis and emphysema （CPFE） syndrome is being increasingly recognized. CPFE is characterized by upper lobe emphysema and lower lobe fibrosis on CT, with normal or mildly abnormal lung volume, significant reduction of dispersion capacity, and hypoxia after exercise. At present, CPFE syndrome is recognized as a unique disease and the exact pathogenesis is unclear. CPFE syndrome is frequently complicated with pulmonary hypertension, lung cancer, and acute lung injury, with poorer prognosis compared with single pulmonary fibrosis and emphysema. Currently, there is no specific therapy of CPFE syndrome. This article reviewed the research progresses on CPFE syndrome.