Hage René, Frauenfelder Thomas, Clarenbach Christian F, Schuurmans Macé M
Division of Pulmonology, University Hospital Zurich, Zurich, Switzerland.
Faculty of Medicine, University of Zurich, Zurich, Switzerland.
J Thorac Dis. 2024 Nov 30;16(11):7290-7299. doi: 10.21037/jtd-24-1200. Epub 2024 Nov 21.
Combined pulmonary fibrosis and emphysema (CPFE) patients generally have a poorer prognosis compared to those with either pulmonary fibrosis or chronic obstructive pulmonary disease (COPD)/emphysema alone, as demonstrated by several studies. Our study aimed to identify undiagnosed CPFE cases within a lung transplantation cohort initially diagnosed with interstitial lung fibrosis or COPD/emphysema. It was hypothesized that the patient cohort might contain overlooked CPFE cases, and CPFE patients would exhibit a higher incidence of pre-transplant pulmonary hypertension and post-transplant chronic lung allograft dysfunction (CLAD).
This is a single-center, retrospective cohort study conducted at the University Hospital of Zurich, Switzerland. Expert review of pre-transplant computed tomography (CT) scans by a thoracic radiologist aimed to identify undiagnosed CPFE cases. Diagnostic criteria followed established guidelines, requiring both emphysema and pulmonary fibrosis on CT imaging. A total of 133 consecutive adult bilateral lung transplantations were performed for patients with interstitial lung disease, emphysema, or both (January 1, 2013, to December 31, 2021). A total of 113 patients could be analyzed, 20 patients were excluded because of missing data. The study analyzed pulmonary function tests, pre-transplant pulmonary hypertension screening, and CLAD staging in CPFE and non-CPFE patients. Primary outcome was the incidence of CPFE among lung transplantation candidates initially diagnosed with interstitial lung fibrosis or COPD/emphysema. Secondary outcomes were comparison of pulmonary function, diffusing capacity, invasively measured pulmonary hypertension, and CLAD stages between CPFE and non-CPFE patients.
Based on pre-transplant CT scans, out of 113 patients, 12 (10.6%) were reclassified as previously undiagnosed CPFE, 49 (43.4%) as non-CPFE fibrosis, and 50 (44.2%) as non-CPFE emphysema. A solitary patient (0.9%) was classified as having both fibrosis and emphysema but did not meet the criteria of CPFE. One additional patient (n=1, 0.9%) exhibited a tree-in-bud pattern. Prior to the radiological review, none of the patients had been classified as CPFE and had instead received a radiological diagnosis of COPD, emphysema, sarcoidosis, interstitial pulmonary fibrosis (IPF) or hypersensitivity pneumonitis (HP).
The study confirmed undiagnosed CPFE cases in 12 (10.6%) in the lung transplantation cohort, suggesting potential underdiagnosis or misclassification. Objective analysis revealed similarities in lung function, diffusing capacity, pulmonary hypertension and CLAD between CPFE and non-CPFE groups. Further exploration is warranted to understand CPFE's diagnostic nuances and clinical implications in lung transplantation.
多项研究表明，与单纯患有肺纤维化或慢性阻塞性肺疾病（COPD）/肺气肿的患者相比，合并肺纤维化和肺气肿（CPFE）的患者预后通常更差。我们的研究旨在识别最初被诊断为间质性肺纤维化或COPD/肺气肿的肺移植队列中未被诊断出的CPFE病例。据推测，该患者队列中可能存在被忽视的CPFE病例，并且CPFE患者移植前肺动脉高压和移植后慢性肺移植功能障碍（CLAD）的发生率会更高。
这是一项在瑞士苏黎世大学医院进行的单中心回顾性队列研究。由胸放射科医生对移植前计算机断层扫描（CT）扫描进行专家评估，旨在识别未被诊断出的CPFE病例。诊断标准遵循既定指南，要求CT成像上同时存在肺气肿和肺纤维化。对133例因间质性肺疾病、肺气肿或两者兼有而接受连续成人双侧肺移植的患者进行了研究（2013年1月1日至2021年12月31日）。总共113例患者可进行分析，20例患者因数据缺失被排除。该研究分析了CPFE和非CPFE患者的肺功能测试、移植前肺动脉高压筛查以及CLAD分期。主要结局是最初被诊断为间质性肺纤维化或COPD/肺气肿的肺移植候选者中CPFE的发生率。次要结局是比较CPFE和非CPFE患者之间的肺功能、弥散能力、有创测量的肺动脉高压以及CLAD分期。
根据移植前CT扫描，在113例患者中，12例（10.6%）被重新分类为先前未被诊断出的CPFE，49例（43.4%）为非CPFE纤维化患者，50例（44.2%）为非CPFE肺气肿患者。有1例患者（0.9%）被分类为同时患有纤维化和肺气肿，但不符合CPFE的标准。另有1例患者（n = 1，0.9%）表现出树芽征。在放射学评估之前，没有患者被分类为CPFE，而是被诊断为COPD、肺气肿、结节病、间质性肺纤维化（IPF）或过敏性肺炎（HP）。
该研究证实了肺移植队列中有12例（10.6%）未被诊断出的CPFE病例，提示可能存在诊断不足或分类错误的情况。客观分析显示CPFE组和非CPFE组在肺功能、弥散能力、肺动脉高压和CLAD方面存在相似之处。有必要进一步探索以了解CPFE在肺移植中的诊断细微差别和临床意义。
