Objective To improve the knowledge and diagnostic accuracy of combined pulmonary fibrosis and emphysema (CPFE) syndrome in connective tissue diseases (CTD) by summarizing the clinical characteristics of 20 CTD patients with CPFE and reviewing literatures. Methods The medical records of 20 CTD patients with CPFE from January 2011 to June 2015 were retrospectively analyzed. Results There were 11 males and 9 females. The average age was 47 years. Among them, 4 patients were smokers and 15 patients were nonsmokers. The average duration of CTD was 3.5 years with an average onset age of 41 years. Respiratory symptoms were reported in 17 patients and Velcro rale was found in 9 patients; The most common type of CTD disease in these 20 patients was inflammatory myopathy (9 patients, 45%) followed by systemic sclerosis (SSc) (4 patients, 20%). High resolution computerized tomography of lung showed typical radiological features of CPFE containing fibrosis lesions predominantly distributed in the subpleural (14 patients) and basal (18 patients) parts and emphysema mainly located in upper zones. Relatively normal results of lung volume and ventilation function, and markedly reduced carbon monoxide transfer capacity were observed. One patient was confirmed with pulmonary hypertension and 1 patient died from severe inflammation and acute respiratory distress syndrome. Conclusions The CPFE syndrome can be identified in CTD patients as an entity with male predominance, especially among patients with inflammatory myopathy and SSc. Higher risk of secondary pulmonary hypertension and acute lung injury in these patients may increase mortality. Early differentiation of CPFE from pure interstitial lung disease in CTD patients could be helpful in improving prognosis.
ObjectiveTo study the possbility of using intranasal instillation of fine particulate matter (PM2.5) combined with inhalation of ozone (O3) to establish mouse model of combined pulmonary fibrosis and emphysema (CPFE), and to provide a reference for the establishment of CPFE model.MethodsMale C57/BL6 mice were divided randomly into phosphate buffer saline (PBS) intranasal instillation+air inhalation group, PBS intranasal instillation+O3 inhalation group and PM2.5 intranasal instillation+O3 inhalation group, with 8 mice in each group. The mice were intranasally instilled with PBS or PM2.5 suspension (7.8 mg/kg) followed by air or ozone inhalation 24 hours later, twice a week over 8 weeks. Lung function, bronchoalveolar lavage fluid (BALF) cell counts and classification were detected, the pathological changes of lung tissues in hematoxylin-eosin staining were observed, including inflammation scores and mean linear intercept (Lm). The thickness of collagen deposition in subepithelium was measured in lung tissues in Masson staining, and simultaneously hydroxyproline contents in lung tissues were determined.ResultsCompared to PBS instillation+air inhalation group, inspiratory capacity (IC), total lung capacity (TLC) and chord compliance (Cchord) were increased, FEV25 (the forced expiratory volume at 25 ms)/FVC (forced vital capacity) was decreased, total cell counts in BALF, Lm and lung inflammatory scores were increased, the thickness of the subepithelial collagen layer (SEc/Pbm) or hydroxyproline contents was not changed in PBS instillation +O3 inhalation group; IC was decreased, functional residual capacity (FRC) was increased, TLC was increased, Cchord was decreased, FEV25/FVC and FEV50 (the forced expiratory volume at 50 ms)/FVC were decreased, total cell counts in BALF, Lm, lung inflammatory scores, SEc/Pbm and hydroxyproline contents were increased in PM2.5 instillation+O3 inhalation group. Compared to PBS instillation+O3 inhalation group, IC was decreased, FRC was increased, Cchord was decreased, FEV25/FVC and FEV50/FVC were decreased, total cell counts in BALF, Lm, lung inflammatory scores, SEc/Pbm and hydroxyproline contents were increased in PM2.5 instillation +O3 inhalation group.ConclusionCPFE mouse model can be successfully established by PM2.5 intranasal instillation combined with ozone inhalation for consecutive 8 weeks.
ObjectivesTo compare the clinical features of combined pulmonary fibrosis and emphysema (CPFE) and idiopathic pulmonary fibrosis (IPF).MethodsEighty-three patients diagnosed as CPFE or IPF for the first time were retrospectively analyzed from June 2014 to July 2018 in Nanjing Drum Tower Hospital, including 47 patients in the CPFE group and 36 in the IPF group. The demographic characteristics, clinical manifestations, pulmonary function, cardiac ultrasound, blood gas analysis and prognosis of the two groups were compared.ResultsThe proportion of smokers in the CPFE group was higher than IPF group (P<0.05), but dyspnea was lower (P<0.05). The FVC, FVC%pred, FEV1, FEV1%pred and VC% of the CPFE group were higher than IPF group (P<0.05), while FEV1/FVC%pred in the IPF group was higher than CPFE group (P<0.05). DLCO/VA%pred of CPFE group decreased more significantly than IPF group (P<0.05), RV/TLC%pred of CPFE group increased annually, while decreased annually in IPF group (P<0.01). The RV%pred of CPFE increased annually, while that of IPF group decreased annually (P<0.05). There was no significant difference in arterial oxygen pressure and pulmonary artery pressure between the two groups. As for prognosis, the 1- and 3-year survival rate of the CPFE group were 87.9% and 73.8% respectively, those of the IPF group were 84.1% and 65.8% respectively, and no significantly difference was observed between two groups (P=0.95).ConclusionsCompared with IPF, patients with CPFE usually have more smokers, less proportion of dyspnea, almost normal lung volume, more rapidly decreased DLCO/VA%pred, and no significant difference in prognosis.
ObjectiveBased on real-word data, and compared with two common chronic respiratory diseases, interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD), this case-control study plans to investigate the risk factors and clinical characteristics of patients with combined pulmonary fibrosis and emphysema syndrome (CPFE).MethodsA retrospective case-control study was carried out to screen the clinical data of 96 patients with CPFE, 133 patients with COPD and 164 patients with ILD, analyze their demographics, clinical data, complications and related clinical indicators. Univariate analysis was used to compare the differences among the three groups, and multivariate logistic analysis was used to screen for risk factors.ResultsAll three groups were in old age with the average age of above 71 years. In terms of male ratio and smoking rate, the CPFE group (93.8%, 85.4%) was higher than the ILD group (75.0%, 64.0%), but there was no significant difference when compared with the COPD group (90.2%, 82.0%). Regarding comorbid disease, the proportion of connective tissue disease (CTD) in the CPFE group (10.4%) and the ILD group (13.4%) was higher than that in the COPD group (1.5%). The proportion of hyperlipidemia in the CPFE group (8.3%) was higher than that in the COPD group (1.5%) and the ILD group (1.2%). There were differences in the abnormal proportion of antinuclear antibody among the three groups, but no significant difference was found when compared with the CPFE group alone. The CPFE group (46.9%, 12.5%) and the ILD group (54.9%, 9.8%) were significantly higher than the COPD group (34.6%, 2.3%) in terms of carcinoembryonic antigen (CEA) abnormal proportion and cancer rate. In terms of the prevalence of pulmonary hypertension, the CPFE group (41.7%) > the COPD group (33.1%) > the ILD group (32.9%) was shown, but no statistical significance was found among the three groups.ConclusionsMale and smoking are not only risk factors for COPD but also for CPFE. At the same time, the suffering of CPFE may be affected by immune factors and hyperlipidemia. The proportion of CPFE patients complicated with cancer and CEA abnormalities is higher than COPD patients. The severity of pulmonary hypertension in CPFE patients is significantly higher than the other two diseases.
Objective To study the prevalence of combined pulmonary fibrosis and emphysema (CPFE) in a community-based lung cancer screening program in Shanghai. Methods From June 2018 to July 2019, eligible participants who were assessed through a high-risk lung cancer questionnaire in Xuhui, Shanghai underwent low-dose computed tomography of the lungs. The suspected CPFE patients were invited to provide medical history and blood for analysis, and received high-resolution CT (HRCT) scanning for confirmation. Results Of the 15 cases of suspected CPFE from a total of 4478 participants in which 1704 males and 2774 females, 4 declined further examination and 11 received further examine. Eight subjects were confirmed as CPFE, and all were male, of whom two were ex-smokers and six were active smokers. These CPFE patients had cough, chest tightness and dyspnea. There were 3 cases of centrilobular emphysema, 2 cases of paraseptal emphysema, 1 case of panlobular emphysema and 2 cases of mixed emphysema. There were 2 cases of usual interstitial pneumonia, 3 cases of non-specific interstitial pneumonia, 2 cases of airspace enlargement with fibrosis and 1 case of unclassifiable smoking-related interstitial fibrosis. The KBILD scores were 61.7±7.5 and mMRC scores were 1.5±0.8. Serum Krebs von den Lungen-6 concentration was (380.75±212.05)U/mL. Lung function test showed normal or mild restrictive ventilatory function, and mild-moderate impairment in diffusion capacity. Conclusions The prevalence of CPFE is 1.79‰ in a community-based lung cancer screening population, and is 4.69‰ in male lung cancer screening population.