Nasim Faria, Moua Teng
Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.
ERJ Open Res. 2020 Dec 14;6(4). doi: 10.1183/23120541.00521-2020. eCollection 2020 Oct.
Combined pulmonary fibrosis and emphysema (CPFE) is characterised by upper lobe emphysema and lower lobe fibrosis. Our study aim was to determine the incident risk, presenting characteristics and outcome of lung cancer diagnoses in a cohort of CPFE patients over time.
We conducted a retrospective cohort study assessing patients with radiological CPFE followed over a median of 76 months (range 1-237 months). Interval development of lung cancer and clinicopathological characteristics of those with and without lung cancer were compared and survival analysis performed.
Lung cancer occurred in 26 (11.6%) out of 230 CPFE patients, dominated by nonsmall cell lung cancer (88%, n=23) with squamous cell carcinoma comprising the majority (57%, n=13). There was a predominance of lower lobe (62%) and subpleural (64%) radiological presentation. Survival was reduced for the whole cohort by lung cancer even after adjusting for covariables of age, sex, smoking pack-years, presenting forced vital capacity and radiological honeycombing. Univariable predictors of increased mortality after lung cancer diagnosis included honeycombing (hazard ratio (HR) 3.03, 95% CI 1.16-7.91; p=0.02) and later stage presentation (HR 4.77, 95% CI 1.8-14.94; p=0.001), with those able to undergo surgical resection having better survival (HR 0.29, 95% CI 0.09-0.87; p=0.02).
Lung cancer occurred in 26 (11.6%) out of 230 CPFE patients and was dominated by squamous cell carcinoma presenting in a lower lobe peripheral distribution. Surgical resection appeared to improve survival in selected patients with earlier stage disease. Further studies are needed to develop a relevant screening programme for CPFE patients.
合并肺纤维化和肺气肿（CPFE）的特征为上叶肺气肿和下叶纤维化。我们的研究目的是确定CPFE患者队列中肺癌诊断的发病风险、呈现特征及转归情况。
我们进行了一项回顾性队列研究，评估经影像学诊断为CPFE的患者，随访时间中位数为76个月（范围1 - 237个月）。比较肺癌的间隔期发生情况以及有肺癌和无肺癌患者的临床病理特征，并进行生存分析。
230例CPFE患者中有26例（11.6%）发生肺癌，以非小细胞肺癌为主（88%，n = 23），其中鳞状细胞癌占多数（57%，n = 13）。放射学表现以下叶（62%）和胸膜下（64%）为主。即使在对年龄、性别、吸烟包年数、就诊时的用力肺活量和放射学蜂窝状改变等协变量进行校正后，肺癌仍使整个队列的生存率降低。肺癌诊断后死亡率增加的单变量预测因素包括蜂窝状改变（风险比（HR）3.03，95%可信区间1.16 - 7.91；p = 0.02）和晚期表现（HR 4.77，95%可信区间1.8 - 14.94；p = 0.001），能够接受手术切除的患者生存率更高（HR 0.29，95%可信区间0.09 - 0.87；p = 0.02）。
230例CPFE患者中有26例（11.6%）发生肺癌，以在下叶周边分布的鳞状细胞癌为主。手术切除似乎可改善部分早期疾病患者的生存率。需要进一步研究为CPFE患者制定相关筛查方案。
