肺纤维化合并肺气肿相关肺动脉高压新视点  

A new perspective on combined pulmonary fibrosis and emphysema associated pulmonary hypertension

作　　者：张甲燚 陈玲 何梦钰 张群 孔辉 解卫平 Zhang Jiayi;Chen Ling;He Mengyu;Zhang Qun;Kong Hui;Xie Weiping(Department of Respiratory and Critical Care Medicine,the First Affiliated Hospital with Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院呼吸与危重症医学科,南京210029

出　　处：《国际呼吸杂志》2024年第5期605-610,共6页International Journal of Respiration

基　　金：国家自然科学基金(82000061);中国博士后科学基金面上项目(2020M671396)。

摘　　要：肺纤维化合并肺气肿(CPFE)相关肺动脉高压(PH)属于第三大类PH,有别于其他慢性缺氧相关性PH,进展快、预后差、5年生存率低,因临床尚无CPFE相关PH的标准诊断和治疗指南,此类患者易漏诊、误诊。CPFE是一种阻塞和限制性通气功能障碍共存的临床综合征,其影像学具有中上肺野肺气肿、肺大疱,下肺野纤维化的特点,表现为厚壁囊性病变的独特表现。本文梳理了CPFE相关PH的临床特征、发病机理、处理策略,以提高临床医师对CPFE相关PH的认识,早期识别,改善患者预后。Combined pulmonary fibrosis and emphysema(CPFE)associated pulmonary hypertension(PH)is classified as the third major type of PH.Differing from other types of chronic hypoxia associated PH,CPFE patients with PH have poor prognosis,rapid disease progression and low 5-year survival.Due to the lack of standardized diagnostic and treatment guidelines for CPFE associated PH in clinical practice,missed diagnosis or delayed diagnosis and treatment easily occurs.CPFE is a clinical syndrome characterized by the coexistence of obstructive and restrictive ventilation dysfunction.Its imaging features include emphysema in the upper and middle lung fields,bullae in the lungs,and fibrosis in the lower lung fields,presenting as a unique manifestation of thick-walled cystic lesions.This article summarizes the clinical characteristics,pathogenesis,and management strategies of CPFE associated PH,aiming to improve the clinical understanding of CPFE associated PH and favor the early identification and prognosis.

关 键 词：高血压 肺性 肺弥散能力 肺纤维化合并肺气肿 厚壁囊性病变 

分 类 号：R563[医药卫生—呼吸系统] R544.1[医药卫生—内科学]