Combined Pulmonary Fibrosis And Emphysema:Report Of Seven Cases And Literature Review

Objective:1.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.2.Based on the review of related literature comprehensively, we describe the understanding of combined pulmonary fibrosis and emphysema (CPFE) syndrome in nearly a decade from both domestic and overseas professionals. Method:1. Collect qianfoshan hospital of Shandong university2010-2012of hospitalization in7CPFE medical records of patients.2. 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.3. Summarize the clinical characteristics of combined pulmonary fibrosis and emphysema (CPFE) syndrome based on the related clinical literature. Results:1.The subject consists of7patients (6males and1females), aged from62to84yea rs, with the average of77.4years. Six of them were smokers, the other one had been working as transplanting wine slag for more than40years. All patients had a dyspnea on exertion and basal crackles COuld be heard at the lower fields of their both sides of lung.2.The pulmonary function demonstrated that FEV1%pred was73%(39%～98%), FVC%pred was73%(54%～101%),FEV1/FVC%was81%(61%～96%),VC%pred was79%(57%～86%),TLC%pred was85%(53%～110%),RV/TLC%was41%(21%～58%).DLco%pred was46%(20%～58%),DLco/VA%was535(19%～98%)3.There are4cases with pulmonary hypertension with an average of45mmHg.Of2cases have an increase at neuron specific enolase in the lung cancer biomarkers.4.Emphysema predominantly at the upper lobes and reticular opacities, honeycombing and traction bronchiectasis in the lower lobes can be seen in the high resolution computerized tomography(HRCT).Conelusions:1.The CPFE syndrome typically occurs in mal e smokers.2.Clinical characteristics include the presence of dyspnea,pulmonary hypertension, and hypoxemia.3.Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange.4.Radiographic characteristics include the presence of upper-lobe emphysema and lower-lobe pulmonary fibrosis.5.This complication of this syndrome frequently is pulmonary hypertension,acute lung injury and lung cancer.