Research Of Combined Pulmonary Fibrosis And Emphysema

Objective:1.To retrospectively analyse the clinical features, smoking history, pulmonary function,pulmonary artery pressure, mortality and radiological appearance in patients with combined pulmonary fibrosis and emphysema(CPFE).2.To compare the characteristics of CPFE vs emphysematous COPD and CPFE vs idiopathic pulmonary fibrosis(IPF).Research method:This is a clinical retrospective case and control study. We designed three different groups of hospitalized patients since2000to2012at Beijing Hospital:cases of CPFE, cases of emphysematous COPD, cases of IPF, and formulate the standards of enrollment and elimination. We selected patients who had full informations. Patients diagnosed with CPFE according to the chest high-resolution computed tomography(HRCT) findings (with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs). The diagnosis of COPD refers to the diagnostic and treatment guidelines of GOLD (Recension of2011) and IPF refers to the diagnostic and management guidelines of ATS/ERS/JRS/ALAT (Recension of2011). We reviewed the clinical features, smoking history,pulmonary function, pulmonary artery pressure, mortality and radiological appearance in the three different groups.Results:1.55CPFE cases were enrolled in this study.All patients but one were male. Age of this group was84.11±11.00. There were38smokers (65.45%), smoking index was24.39±20.28pack-years. Dyspnoea was presented in41patients (74.5%) and basal crackles were heard in43patients (78.2%).16patients were admitted to the hospital as pulmonary fibrosis while39patients were as COPD.12patients came to pass lung cancer and the mean time from diagnosis of CPFE to cancer was3.56±2.99years.28patients were dead and the main reason was sepsis shock (46.4%).7autopsy cases showed centrilobular emphysema and bullae at the upper zone, while organized pneumonia at the lower zone. Only one case was UIP. Pulmonary hypertension was present in29.1%of patients(59.31±11.79mmHg).Blood gas analysis(mean±SD):PaO2 67.58±13.26mmHg;PaCO238.75±8.7mmHg;PA-aO258.76±51.18mmHg. Pulmonary function tests were as follows:FVC%Pre88.83±22.42%; FEV1%Pre79.52±20.94%; FEV1/FVC%67.87±12.87%; TLC%Pre105.43±27.91%; RV/TLC%49.8±11.24; DLco%Pre72.54±27.66。2.55COPD cases and40IPF cases were enrolled in as control groups.4patients came to pass lung cancer in IPF group and the mean time from diagnosis of IPF to cancer was3.25±2.22years. FEV1/FVC%(t=-5.069,P=0.000), DLco(t=-4.573, P=0.000),PA-aO2(Z=-3.142,P=0.001)andFEV1%Pre(Z=-5.149,P=0.000)had statistical significant difference between group CPFE and group COPD. FEV1/FVC%(t=-4.056,P=0.000),TLC%Pre(t=4.644,P=0.000),PA-aO2(Z=-3.954,P=0.000)and points of pulmonary fibrosis(Z=-6.567,P=0.000) had statistical significant difference between group CPFE and group IPF.Conclusion:1.CPFE syndrome is a distinct clinical entity. Smoking is the principal risk factor for CPFE. Most of the patients have dyspnoea.2. A high prevalence of pulmonary artery hypertension and lung cancer is seen in CPFE syndrome.3. Patients with CPFE syndrome can present with a normal or nearly normal lung volume but a remarkable impairment in gas exchange and has a correlation with the proportion of emphysema and Pulmonary fibrosis on HRCT.