Combined pulmonary fibrosis and emphysema syndrome:a report of seven cases and literature review

Abstract

Objective To investigate the general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of patients with combined pulmonary fibrosis and emphysema (CPFE) syndrome.Methods The general,clinical,pulmonary function,pulmonary arterial pressure,the biomarker of lung cancer,radiological features,and prognosis of seven patients diagnosed with CPFE syndrome were retrospectively analyzed.Results The subject consisted of seven patients (six males and one female),aged from 62 to 84 years old,with the average of 77.4 years old.Six of them were smokers,the other one had been working as transplanting wine slag for more than 40 years.All patients had a dyspnea on exertion and basal crackles could be heard at the lower fields of their both sides of lung.The pulmonary function demonstrated that FEV1％ pred was 73 ％ (39 ％-98％),FVC％pred was 73％(54％-101％),FEV1/FVC％ was 81％(61％-96％),VC％pred was 79％ (57％-86％),TLC％pred was 85％ (53％-110％),RV/TLC％ was 41％ (21％-58％).DLCO％ pred was 46％(20％-58％),DLCO/VA was 53％ (19％-98％).Four cases had pulmonary hypertension with an average of 45 mmHg.The level of neuron specic enolase increased in two cases.High resolution computerized tomography showed emphysema predominantly at the upper lobes and reticular opacities,honeycombing and traction bronchiectasis in the lower lobes.Conclusions The CPFE syndrome typically occurs in male smokers,and the mortality is associated with this.Clinical characteristics include the presence of dyspnea,pulmonary hypertension,and hypoxemia.Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange.Radiographic characteristics include the presence of upper-lobe emphysema and lower-lobe pulmonary fibrosis.The complication of this syndrome frequently is pulmonary hypertension,acute lung injury,and lung cancer.Further studies are needed to explore the pathogenesis,treatment,and prognosis of CPFE. Key words: Pulmonary fibrosis; Emphysema; Combined pulmonary fibrosis and emphysema; Smoking ; Pulmonary function