肺纤维化合并肺气肿综合征的临床特点及诊疗研究进展

Clinical characteristics and research advances in diagnosis and treatment of combined pulmonary fibrosis and emphysema syndrome

肺纤维化合并肺气肿(CPFE)综合征是一种不同于单纯肺气肿或肺纤维化的新型临床综合征,该病在影像学上表现为上肺的肺气肿以及下肺的肺纤维化。目前其发病原因不明,国内外缺乏统一的诊疗指南,并发症多且预后较差。因此,如何对CPFE患者进行早期识别并干预已成为呼吸科医生面对的一大难题。本文就CPFE的病因及发病机制、临床表现、辅助检查、诊断、治疗、并发症、预后等方面进行综述,以期为该病的临床诊治提供参考。

Combined Pulmonary Fibrosis and Emphysema(CPFE)syndrome is a new clinical syndrome distinct from pure emphysema or pulmonary fibrosis,which is characterized by emphysema in the upper lungs and fibrosis in the lower lungs on imaging.The etiology of CPFE is currently unknown,and there is a lack of unified diagnostic and treatment guidelines both in China and globally.The syndrome is associated with multiple complications and poor prognosis.Therefore,early i-dentification and intervention of CPFE patients have become a major challenge for respiratory physicians.This article pro-vides a comprehensive review of the etiology,pathogenesis,clinical manifestations,auxiliary examinations,diagnosis,treat-ment,complications,and prognosis of CPFE,aiming to provide a reference for the clinical diagnosis and treatment of this disease.