Feng Xiaoyi, Zeng Wenjing, Lv Xiafei, Liang Binmiao, Ou Xuemei
Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Department of Pulmonary and Critical Care Medicine, West China Hospital, Sichuan University, Chengdu 610041, China.
J Clin Med. 2025 May 30;14(11):3862. doi: 10.3390/jcm14113862.
Idiopathic pulmonary fibrosis (IPF) and emphysema often coexist in patients with lung cancer (LC), forming a syndrome with combined pulmonary fibrosis and emphysema (CPFE). The three share the pathogenic mechanisms of smoking, chronic inflammation, and oxidative stress. The clinical management of CPFE patients is challenging, but its impact on tumor characteristics, acute exacerbation (AE), and prognosis is still controversial. The purpose of this study was to clarify the effect of CPFE on tumor biological behavior, AE risk, and survival outcome in patients with IPF-LC so as to optimize individualized treatment strategies. This was a retrospective and single-center study. Newly diagnosed LC patients with IPF, COPD, and normal lungs were recruited in the west China hospital. Patients with IPF were further categorized into CPFE-LC and isolated IPF-LC groups based on the presence of emphysema. Clinical and tumor features, lung function parameters, and prognosis were obtained and compared. Patients with IPF and LC were more common in older men and heavy smokers. IPF-associated tumors had a higher proportion of carrying EGFR wild-type, occurring in the lower lobe of the lung and developing adenocarcinoma and squamous cell carcinoma. Among IPF-LC patients, 68.2% (103/151) met CPFE criteria. Pulmonary function tests demonstrated preserved VC% but significantly reduced FEV1/FVC in CPFE versus non-emphysema IPF (76.3% vs. 80.7%, = 0.004), alongside elevated CPI and impaired DLCO. CPI ≥ 40 (HR = 2.087, 95%CI: 1.715-6.089, = 0.012), combined with COPD (HR = 2.281, 95%CI: 1.139-4.569, = 0.040), isolated IPF (HR = 5.703, 95%CI: 2.516-12.925, < 0.001), and CPFE (HR = 6.275, 95%CI: 3.379-11.652, < 0.001), were independent prognostic risk factors in LC patients. The incidence of treatment-induced AEs (49.5% vs. 29.2%, = 0.038) and AE-related mortality (28.0% vs. 11.8%, = 0.045) were significantly higher in the CPFE group than in the isolated IPF group. Logistic regression analysis showed that CPFE (OR: 3.494, 95%CI: 2.014-6.063, = 0.001) was independently associated with the risk of AE-related mortality in patients with LC and IPF. Compared to LC patients with solely IPF, the presence of emphysema had no significant impact on overall survival, but CPFE increased the risk of treatment-triggered AE and was associated with AE-related mortality. In patients with LC, CPFE with AEs had a worse prognosis than IPF with AEs.
特发性肺纤维化（IPF）和肺气肿在肺癌（LC）患者中常并存，形成一种合并肺纤维化和肺气肿（CPFE）的综合征。三者具有吸烟、慢性炎症和氧化应激等共同致病机制。CPFE患者的临床管理具有挑战性，但其对肿瘤特征、急性加重（AE）和预后的影响仍存在争议。本研究旨在阐明CPFE对IPF-LC患者肿瘤生物学行为、AE风险和生存结局的影响，以优化个体化治疗策略。 这是一项回顾性单中心研究。在中国西部医院招募了新诊断的合并IPF、慢性阻塞性肺疾病（COPD）和肺功能正常的LC患者。根据是否存在肺气肿，将IPF患者进一步分为CPFE-LC组和孤立性IPF-LC组。获取并比较临床和肿瘤特征、肺功能参数及预后情况。 IPF合并LC在老年男性和重度吸烟者中更为常见。IPF相关肿瘤携带表皮生长因子受体（EGFR）野生型的比例更高，发生于肺下叶，且多为腺癌和鳞癌。在IPF-LC患者中，68.2%（103/151）符合CPFE标准。肺功能测试显示，与非肺气肿IPF患者相比，CPFE患者的肺活量百分比（VC%）保持正常，但第一秒用力呼气容积与用力肺活量比值（FEV1/FVC）显著降低（76.3%对80.
