Combined pulmonary fibrosis and emphysema: a retrospective analysis of clinical characteristics, treatment and prognosis

BACKGROUND: Combined pulmonary fibrosis and emphysema (CPFE) is increasingly acknowledged as a separate syndrome with distinct clinical, physiological and radiological characteristics. We sought to identify physiologic and radiographic indices that predict mortality in CPFE. METHODS: Data on clinical characteristics, pulmonary function, high-resolution computed tomography (HRCT) and treatment were compared between patients with usual interstitial pneumonia (UIP) plus emphysema (CPFE group) and those with IPF alone (IPF group). Composite physiologic index (CPI) and HRCT scores at diagnosis and during follow-up were assessed. RESULTS: CPFE group (N = 87) was characterized by the predominance of males and smokers, who were less likely to have viral infection prior to the diagnosis, and display basal crackles, finger clubbing and wheeze, as compared to that in the IPF group (N = 105). HRCT and CPI scores increased over time in both groups. Moreover, CPFE group had a poorer prognosis, lower 5-year survival rate (43.42 % vs. 65.56 %; P < 0.05), and higher mortality (39.47 % vs. 23.33 %; P < 0.05) as compared to that in the IPF group. All CPFE patients received oxygen therapy, antibiotics and oral N-acetylcysteine; > 50 % received bronchodilators, 40 % received corticosteroids and 14 % needed noninvasive mechanical ventilation. On survival analyses, pulmonary arterial hypertension (PAH) and ≥ 5-point increase in CPI score per year were predictors of mortality in the CPFE group (hazard ratio [HR]: 10.29, 95 % Confidence Interval [CI]: 2.69–39.42 and HR: 21.60, 95 % CI: 7.28–64.16, respectively). CONCLUSION: Patients with CPFE were predominantly male and smokers and exhibited distinct clinical, physiological and radiographic characteristics. They had a poorer prognosis than IPF. PAH and ≥ 5-point increase in CPI score per year were predictors of mortality in these patients. Future studies are needed to identify the optimal treatment approach to CPFE.