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Original Research: Diffuse Lung Disease |

Clinical Features and Outcomes in Combined Pulmonary Fibrosis and Emphysema in Idiopathic Pulmonary FibrosisCombined Pulmonary Fibrosis and Emphysema

Christopher J. Ryerson, MD; Thomas Hartman, MD; Brett M. Elicker, MD; Brett Ley, MD; Joyce S. Lee, MD; Marta Abbritti, MD; Kirk D. Jones, MD; Talmadge E. King, Jr, MD; Jay Ryu, MD, FCCP; Harold R. Collard, MD, FCCP
Author and Funding Information

From the Department of Medicine (Dr Ryerson), University of British Columbia, Vancouver, BC, Canada; Department of Radiology (Dr Hartman), Department of Medicine (Dr Ryu), Mayo Clinic, Rochester, MN; Department of Radiology (Dr Elicker), Department of Medicine (Drs Ley, Lee, King, and Collard), and Department of Pathology (Dr Jones), University of California San Francisco, San Francisco, CA; and the Pulmonary Institute and Respiratory Intensive Care Unit (Dr Abbritti), S. Maria della Misericordia Hospital, Perugia, Italy.

Correspondence to: Christopher J. Ryerson, MD, St. Paul’s Hospital Ward 8B, 1081 Burrard St, Vancouver, BC, V6Z 1Y6, Canada; e-mail: chris.ryerson@hli.ubc.ca


Funding/Support: The authors have reported to CHEST that no funding was received for this study.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;144(1):234-240. doi:10.1378/chest.12-2403
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Background:  Combined pulmonary fibrosis and emphysema (CPFE) is increasingly recognized, but its prevalence and prognosis remain unclear. We sought to determine the prevalence, clinical features, and prognosis of CPFE in idiopathic pulmonary fibrosis (IPF), using a standardized and reproducible definition.

Methods:  Patients with IPF were identified from two ongoing cohorts. Two radiologists scored emphysema and fibrosis severity on high-resolution CT (HRCT) scans. CPFE was defined as ≥ 10% emphysema on HRCT scan. Clinical characteristics and outcomes of patients with CPFE and IPF and those with non-CPFE IPF were compared with unadjusted analysis and then analysis after adjustment for HRCT fibrosis score. Mortality was compared using competing risks regression to handle lung transplantation. Sensitivity analyses were performed using Cox proportional hazards, including time to death (transplantation censored) and time to death or transplant.

Results:  CPFE criteria were met in 29 of 365 patients with IPF (8%), with high agreement between radiologists (κ = 0.74). Patients with CPFE had less fibrosis on HRCT scans and higher FVC, but greater oxygen requirements (P ≤ .01 for all comparisons). Findings were maintained with adjustment for fibrosis severity. Inhaled therapies for COPD were used by 53% of patients with CPFE. There was no significant difference in mortality comparing patients with CPFE and IPF to those with non-CPFE IPF (hazard ratio, 1.14; 95% CI, 0.61-2.13; P = .69).

Conclusions:  CPFE was identified in 8% of patients with IPF and is a distinct, clinical phenotype with potential therapies that remain underutilized. Patients with CPFE and IPF and those with non-CPFE IPF have similar mortality.

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