Chest | 2021
Clinical features and outcomes of combined pulmonary fibrosis and emphysema after lung transplantation.
Abstract
BACKGROUND\nCombined pulmonary fibrosis and emphysema (CPFE) is recognized as a characteristic syndrome of smoking-related interstitial lung disease that has a worse prognosis than idiopathic pulmonary fibrosis (IPF). However, outcomes after lung transplantation for CPFE have not been reported. The aim of this study is to describe the clinical features and outcomes of CPFE after lung transplantation.\n\n\nRESEARCH QUESTION\nWhat are the clinical features and outcomes of CPFE after lung transplantation?\n\n\nSTUDY DESIGN AND METHODS\nThis is a single-center retrospective cohort study of patients with CPFE and IPF who underwent lung transplantation at our center between January 2011 and December 2016. We defined CPFE as ≥ 10% emphysema in the upper lung fields combined with fibrosis on high-resolution computed tomography scan. We characterized the clinical features of patients with CPFE and compared their outcomes after lung transplantation to those with IPF.\n\n\nRESULTS\n27 of 172 (16%) patients with IPF met criteria for CPFE. Severe pulmonary hypertension was present in 16 of 27 (59%) patients with CPFE. On logistic regression analysis, CPFE was significantly associated with primary graft dysfunction (PGD) grade 3 (odds ratio: 3.14, 95% confidence interval [CI]: 1.18-8.37, p=0.02). On competing risk regression analysis, CPFE was associated with acute cellular rejection (ACR) grade ≥ A2, and chronic lung allograft dysfunction (CLAD) (hazard ratio [HR]: 1.89, 95% CI: 1.10-3.25, p=0.02, HR: 1.96, 95% CI: 1.02-3.77, p=0.04, respectively). 5-year survival was 79.0% for the CPFE group and 75.4% for the IPF group, respectively (log rank p = 0.684).\n\n\nINTERPRETATION\nAfter transplant, patients with CPFE were more likely to develop PGD, ACR, and CLAD compared to those with IPF. However, survival was not significantly different between the 2 groups.