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Clinical Characteristics and Outcomes in Veterans with Combined Pulmonary Fibrosis and Emphysema (CPFEE) with and without Concomitant Pulmonary Hypertension

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Conference: 2014 International PHA Conference and Scientific Sessions

Release Date: 06.22.2014

Presentation Type: Abstracts

File Download: 2014 Conference Abstract - Khan

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Background: CPFE carries morbidity and mortality rates that are higher than those in emphysema or pulmonary fibrosis (PF) alone, especially if concomitant pulmonary hypertension (PH) is present. We aimed to identify presentation, progression, disease management and outcomes in veterans with CPFE. A secondary aim was to identify the prevalence of PH and compare characteristics and outcomes in patients with and without this complication.

Methods: We conducted a retrospective analysis at a large academic VA Medical Center (VAMC). We screened records carrying the ICD-9 code for PF and also identified patients with CPFE in pulmonary clinic. CPFE was defined as computed tomography findings of idiopathic PF/usual interstitial pneumonia plus upper lobe emphysema. Exclusion criteria were alternative diagnoses for interstitial lung disease (e.g. connective tissue disease, occupational exposures). We collected demographic, clinical, functional, radiologic, echocardiography and right heart catheterization (RHC) data. PH was defined as right atrial or right ventricular (RV) enlargement, decreased RV function, RV systolic pressure (RVSP) >40 mmHg, or mean pulmonary artery pressure >25 mmHg during RHC. Data are presented as means±SD or percentage (%). P<0.05 was considered statistically significant.

Results: Of 151 patients with an ICD-9 code for PF, 41 CPFE patients were identified (27.2%; all male; age 73.5±0.9 years). All patients were current or former smokers. Of note, 35/41 patients did not carry a diagnosis of CPFE until identified by retrospective chart review. Evidence of PH was present either at diagnosis or developed during follow-up in 18 patients (43.9%). CPFE patients with hemodynamically confirmed PH (n=9) or PH suggested by echocardiography alone (n=9) were diagnosed with CPFE-PH. CPFE-PH patients had a higher incidence of NYHA functional class 3 or 4 symptoms (55.5% vs. 26.1%), lower SpO2 (89.9±1.3% vs. 94.4±0.7%), lower diffusing capacity (32.2±3.8% vs. 45.1±2.9%), lower six-minute-walk-distance (693±93 vs. 934±97 feet), and higher B-type natriuretic peptide levels (413±86 vs. 336±184 pg/ml) than patients with CPFE alone. CPFE-PH patients had more comorbidities, including left heart dysfunction and valvular disease. 3-year mortality in the entire cohort was 63.4%; however, mortality was higher in CPFE-PH patients than in patients without PH (73.3% vs. 56.5%). Causes of death are currently under investigation.

Conclusions: In a large VAMC, CPFE is underdiagnosed and associated with high morbidity and mortality. Evidence of PH is present in almost 50% of patients, and associated with an even higher mortality rate. PH etiology in this group is likely multifactorial and may include a WHO group 2 component. CPFE should be aggressively looked for, as early patient identification and disease-specific interventions may improve outcomes.

Funding: Indiana University and Richard L. Roudebush VA Medical Center