TY - JOUR T1 - Lung Cancer in Combined Pulmonary Fibrosis and Emphysema: A Large Retrospective Cohort Analysis JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00521-2020 SP - 00521-2020 AU - Faria Nasim AU - Teng Moua Y1 - 2020/01/01 UR - http://openres.ersjournals.com/content/early/2020/10/15/23120541.00521-2020.abstract N2 - Background 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 (LC) diagnoses in a cohort of CPFE patients over time.Materials and Methods We conducted a retrospective cohort study assessing patients with radiologic CPFE followed over a median of 76 months (range 1–237). Interval development of LC and clinicopathologic characteristics of those with and without LC was compared and survival analysis performed.Results LC occurred in 26 of 230 (11.6%) CPFE patients dominated by non-small cell lung cancer (88%, N=23) with squamous cell carcinoma comprising the majority (N=13(57%)). There was a predominance of lower lobe (62%) and sub-pleural radiologic presentation (64%). Survival was reduced for the whole cohort by LC even after adjusting for a priori covariables of age, sex, smoking pack years, presenting forced vital capacity (FVC%), and radiologic honeycombing. Univariable predictors of increased mortality after LC diagnosis included honeycombing (HR 3.03 (1.16–7.91), p=0.02) and later stage presentation (HR 4.77 (1.8–14.94) p=0.001), with those able to undergo surgical resection having better survival (HR 0.29 (0.09–0.87) p=0.02).Conclusion LC occurred in 26 of 230 (11.6%) 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 program for CPFE patients.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Nasim has nothing to disclose.Conflict of interest: Dr. Moua has nothing to disclose. ER -