Wang Jennifer M, Araki Tetsuro, Cottin Vincent, Han MeiLan K, Oldham Justin M
Division of Pulmonary and Critical Care Medicine, University of Michigan, Ann Arbor, MI.
Department of Radiology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA.
Chest. 2024 Dec;166(6):1463-1472. doi: 10.1016/j.chest.2024.08.007. Epub 2024 Aug 16.
Combined pulmonary fibrosis and emphysema (CPFE) is an underdiagnosed syndrome in which individuals have variable degrees of pulmonary fibrosis and emphysema. Patients with CPFE have high morbidity, including poor exercise tolerance and increased development of comorbidities. CPFE mortality also seems to outpace that of lone emphysema and pulmonary fibrosis. A major limitation to rigorous, large-scale studies of CPFE has been the lack of a precise definition for this syndrome. A 2022 American Thoracic Society/European Respiratory Society/Japanese Respiratory Society/Latin American Thoracic Association research statement called attention to fundamental gaps in our understanding of CPFE and highlighted the potential use of quantitative imaging techniques to better define CPFE.
Broadly, CPFE has been defined using visual interpretation of chest CT imaging documenting the presence of both emphysema and fibrosis, with varying distributions. When quantitative approaches were involved, varying thresholds of emphysema and fibrosis on imaging have been used across different studies.
This review is structured into three primary themes, starting with early imaging studies, then evaluating the use of quantitative methods and imaging-based thresholds, both in large population studies and single-center cohorts to define CPFE and assess patient outcomes. It concludes by discussing current challenges and how to focus our efforts so that quantitative imaging methods can effectively address the most pressing clinical dilemmas in CPFE.
合并性肺纤维化和肺气肿(CPFE)是一种诊断不足的综合征,患者存在不同程度的肺纤维化和肺气肿。CPFE患者发病率高,包括运动耐量差和合并症发生率增加。CPFE的死亡率似乎也超过单纯肺气肿和肺纤维化。对CPFE进行严格的大规模研究的一个主要限制是缺乏对该综合征的精确定义。2022年美国胸科学会/欧洲呼吸学会/日本呼吸学会/拉丁美洲胸科学会的一份研究声明提请注意我们对CPFE理解上的基本差距,并强调了使用定量成像技术更好地定义CPFE的潜力。
广义上,CPFE是通过对胸部CT成像的视觉解读来定义的,记录肺气肿和纤维化的存在情况及其不同分布。当涉及定量方法时,不同研究对成像上肺气肿和纤维化的阈值设定各不相同。
本综述分为三个主要主题,首先是早期成像研究,然后评估在大型人群研究和单中心队列中使用定量方法和基于成像的阈值来定义CPFE并评估患者预后。最后讨论当前面临的挑战以及如何集中精力,以便定量成像方法能够有效解决CPFE中最紧迫的临床难题。