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

Lijuan Zhang,Fangzhou Chi,Qi Song,Chunli Che,Chunling Zhang,Yupeng Wang,Lu Liu,Fushi Dong

doi:10.1186/s12890-016-0300-7

Abstract

BackgroundCombined 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.MethodsData 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.ResultsCPFE 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).ConclusionPatients 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.

Highlights

Combined pulmonary fibrosis and emphysema (CPFE) is increasingly acknowledged as a separate syndrome with distinct clinical, physiological and radiological characteristics
The composite physiologic index (CPI) that represents a combination of pulmonary ventilation and diffusing capacity, and chest highresolution computed tomography (HRCT) score were obtained to evaluate the extent of disease at diagnosis and at 6 months intervals up to a total of 36 months
Clinical characteristics and outcomes A total of 87 patients were included in the CPFE group (79 males; 69 ± 8.5 years)

Summary

Introduction

Combined pulmonary fibrosis and emphysema (CPFE) is increasingly acknowledged as a separate syndrome with distinct clinical, physiological and radiological characteristics. This study led to the characterization of the syndrome of combined pulmonary fibrosis and emphysema (CPFE), based on radiographic findings of upper lobe emphysema, and lower lobe pulmonary fibrosis (PF). CPFE cases are not uncommon, and the estimated prevalence of emphysema is thought to range between one quarter and one half of all IPF patients [5, 6]. Pulmonary arterial hypertension (PAH) is known to develop in up to half of all patients with CPFE, and has been identified as a key determinant of prognosis [6, 9]. A few studies have suggested pulmonary function parameters and the composite physiologic index (CPI) as being predictors of CPFE prognosis [5]. The therapeutic options for patients with CPFE are limited, and there are no consensus recommendations for treatment of emphysema in the setting of IPF

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