Objective To investigate the prevalence of obstructive sleep apnea hypopnea syndrome ( OSAHS) in patients with idiopathic pulmonary fibrosis ( IPF) and its clinical significance. Methods Sleep quality and breathing disorders were measured by polysomnography and the relationship with lung function was analyzed in 20 IPF patients. Results Thirteen of 20 subjects ( 65% ) had OSAHS as defined by an AHI ≥5 events per hour. Three subjects ( 15% ) had mild OSAHS ( AHI,5 to 20 events per hour) , and 10 subjects ( 50% ) had moderate-to-severe OSAHS ( AHI≥20 events per hour) . The sleep architecture in these patients showed a reduction in sleep efficiency, rapid eye movement ( REM) sleep and slow wave sleep, and a marked sleep fragmentation due to an increased arousal index. The AHI was negatively correlated with FVC% pred ( r =-0.672, P=0.001) and FEV1% pred ( r =-0.659, P=0.002) , and positively correlated with body mass index ( BMI) ( r=0.791, Plt;0.0001) . Conclusions OSAHS is a common comorbidity in IPF. Early treatment of OSAHS may improve quality of life and the prognosis of patients with IPF.
Objective To identify risk factors for fibrosis progression and develop a predictive model in patients with usual interstitial pneumonia (UIP) pattern on CT. Methods We retrospectively enrolled 453 patients with CT-defined UIP or probable UIP, followed for one year. The study endpoint was either meeting progressive pulmonary fibrosis (PPF) criteria or completing one-year follow-up. Clinical features, pulmonary function, and laboratory data were collected. Independent risk factors were identified using logistic regression. Patients were randomly divided into training and validation cohorts at a 7:3 ratio. A nomogram was constructed in the training cohort using R and its performance and clinical utility were evaluated in the validation cohort. Results During one-year follow-up, 160 patients (35.3%) met PPF criteria. Multivariate analysis showed that higher baseline levels of CA19-9 and CA125, as well as the presence of pulmonary hypertension, were independent risk factors for pulmonary fibrosis progression, while a higher percentage of predicted forced vital capacity (FVC) and the presence of emphysema were protective factors. A nomogram model was constructed using these five variables, with the area under the curve (AUC) for predicting fibrosis progression being 0.854 in the training set and 0.817 in the validation set. Clinical decision curve analysis indicated that the model provided the greatest clinical benefit when the threshold probability was between 0.12 and 0.93. Conclusion A nomogram incorporating baseline CA19-9, CA125, FVC % predicted, pulmonary hypertension, and emphysema shows potential for predicting one-year fibrosis progression in UIP patients.
ObjectiveTo systematically investigate the clinical characteristics, risk factors, and prognostic determinants of lung cancer (LC) in patients with combined pulmonary fibrosis and emphysema (CPFE). MethodsA total of 180 CPFE patients admitted to West China Hospital of Sichuan University between December 2010 and December 2022 were collected in this retrospective study to explore the risk factors and prognosis of patients complicated with lung cancer. ResultsCPFE-LC predominantly occurs in elderly male smokers, with squamous cell carcinoma (33.33%) and adenocarcinoma (35.29%) being the most prevalent histological subtypes. Compared with CPFE patients, those with CPFE-LC demonstrated significantly higher proportions of males, current or former smokers, smoking index values, lymphocyte counts, neutrophil-to-lymphocyte ratios, serum albumin levels, fibrinogen levels, FEV1%pred, and VC%pred. However, logistic regression analysis identified only male (OR=19.92, 95%CI 2.83-140.27) and elevated fibrinogen levels (OR=2.09, 95%CI 1.47-2.97) as independent risk factors for lung cancer development in CPFE patients. CPFE-LC patients exhibited significantly shorter survival than CPFE patients (1.67 years vs. 4.92 years, P<0.05). Kaplan-Meier survival analysis revealed no significant difference in survival among CPFE-LC patients with different histological subtypes (squamous cell carcinoma vs. adenocarcinoma vs. others, P>0.05). ConclusionsMale and fibrinogen levels are independent risk factors for lung cancer development in patients with CPFE. Patients with CPFE-LC exhibited significantly shortened survival and poor prognosis. This unfavorable prognosis is closely associated with nutritional status, advanced tumor stage, metastasis status, and treatment modalities, but shows no significant association with histopathological type.
Objective To investigate the characteristics on chest high-resolution computed tomography (HRCT) of patients with interstitial pneumonia with positive myeloperoxidase antineutrophil cytoplasmic antibody (MPO-IP). Methods The extent of fibrosis and subtypes of emphysema on HRCT of MPO-IP patients were retrospectively analyzed and compared with idiopathic pulmonary fibrosis (IPF) cases admitted in the same period. Results From July 2014 to March 2016, 10 patients was diagnosed with IPF and 10 patients was diagnosed with MPO-IP. Emphysema was not different between two groups. Among the MPO-IP patients, 8 patients presented with a usual interstitial pneumonia (UIP) pattern. There existed statistical difference in the bronchial bifurcation level, the fibrosis score of lungs in the MPO-IP patients presented with UIP was lower than that in the IPF patients. Conclusions UIP is the predominant radiologic type of MPO-IP patients. Fibrosis in IPF is more serious than that in MPO-IP with UIP. Paraseptal and centrilobular emphysema are main forms in MPO-IP patients.
Objective To systematically evaluate the effectiveness of N-acetylcysteine (NAC) combined with low-dose glucocorticoid for patients with idiopathic pulmonary fibrosis (IPF). Methods Such databases as The Cochrane Library (Issue 12, 2012), EMbase (January 1974 to July 2012), PubMed (January 1966 to July 2012), CHEST (January 1995 to July 2012), CNKI (January 1994 to July 2012), CBM (January 1978 to July 2012), VIP (January 1989 to July 2012) and WanFang Data (January 1995 to July 2012) were searched to collect the randomized controlled trials (RCTs) about NAC combined with low-dose glucocorticoid versus glucocorticoid alone for IPF patients. Two reviewers independently screened the literature according to the inclusion and exclusion criteria, extracted the data, and assessed the quality, and then the meta-analysis was performed using RevMan 5.1 software. Results A total of seven RCTs including 264 IPF patients were included. The results of meta-analysis demonstrated that, compared with the glucocorticoid used alone, a) NAC combined with low-dose glucocorticoid could significantly improve PaO2 (SMD=0.82 mmHg, 95%CI 0.30 to 1.35, P=0.002) and DLco (SMD=0.59 mmHg, 95%CI 0.16 to 1.03, P=0.008) with a significant difference. b) NAC combined with low-dose glucocorticoid could significantly improve all clinical symptoms (RR=1.56, 95%CI 1.26 to 1.92, Plt;0.000 1). Conclusion NAC combined with low-dose glucocorticoid for IPF patients can significantly improve PaO2, DLco, and the clinical symptoms such as cough, difficulty breathing after activities, cyanosis, and Velcro rales. Due to the quantity and quality limitation of included studies, this conclusion still needs to be further proved by more high quality and double blind RCTs.
Since the beginning of the coronavirus disease 2019 (COVID-19) pandemic at the end of December 2019, more than 85% of the population in China has been infected. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) mainly affects the respiratory system, especially the lungs. The mortality rate of patients with severe infection is high. A percentage of 6% to 10% of patients will eventually develop into COVID-related acute respiratory distress syndrome (CARDS), which requires mechanical ventilation and extracorporeal membrane oxygenation (ECMO) support. Some patients who survive acute lung injury will subsequently develop post COVID-19 pulmonary fibrosis (PCPF). Both fully treated CARDS and severe PCPF are suitable candidates for lung transplantation. Due to the special course, evaluation strategies are different from those used in patients with common end-stage lung disease. After lung transplantation in COVID-19 patients, special treatment is required, including standardized nucleic acid testing for the novel coronavirus, adjustment strategy of immunosuppressive drugs, and rational use of antiviral drugs, which is a big challenge for the postoperative management of lung transplantation. This consensus was evidence-based written and was reached by experts after multiple rounds of discussions, providing reference for assessment and postoperative management of patients with interstitial pneumonia after COVID-19 infection.
ObjectiveAlthough evidence links idiopathic pulmonary fibrosis (IPF) and diabetes mellitus (DM), the exact underlying common mechanism of its occurrence is unclear. This study aims to explore further the molecular mechanism between these two diseases. MethodsThe microarray data of idiopathic pulmonary fibrosis and diabetes mellitus in the Gene Expression Omnibus (GEO) database were downloaded. Weighted Gene Co-Expression Network Analysis (WGCNA) was used to identify co-expression genes related to idiopathic pulmonary fibrosis and diabetes mellitus. Subsequently, differentially expressed genes (DEGs) analysis and three public databases were employed to analyze and screen the gene targets related to idiopathic pulmonary fibrosis and diabetes mellitus. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses were performed by Metascape. In addition, common microRNAs (miRNAs), common in idiopathic pulmonary fibrosis and diabetes mellitus, were obtained from the Human microRNA Disease Database (HMDD), and their target genes were predicted by miRTarbase. Finally, we constructed a common miRNAs-mRNAs network by using the overlapping genes of the target gene and the shared gene. ResultsThe results of common gene analysis suggested that remodeling of the extracellular matrix might be a key factor in the interconnection of DM and IPF. Finally, hub genes (MMP1, IL1R1, SPP1) were further screened. miRNA-gene network suggested that has-let-19a-3p may play a key role in the common molecular mechanism between IPF and DM. ConclusionsThis study provides new insights into the potential pathogenic mechanisms between idiopathic pulmonary fibrosis and diabetes mellitus. These common pathways and hub genes may provide new ideas for further experimental studies.
Objective To clarify the specific clinical predictive efficacy of CT and serological indicators for the progression of connective tissue disease-associated interstitial lung disease (CTD-ILD) to progressive pulmonary fibrosis (PPF). Methods Patients who were diagnosed with CTD-ILD in Chest Hospital of Zhengzhou University Between January 2020 and December 2021 were recruited in the study. Clinical data and high-resolution CT results of the patients were collected. The patients were divided into a stable group and a progressive group (PPF group) based on whether PPF occurred during follow-up. COX proportional hazards regression was used to identify risk factors affecting the progression of CTD-ILD to PPF, and a risk prediction model was established based on the results of the COX regression model. The predictive efficacy of the model was evaluated through internal cross-validation. Results A total of 194 patients diagnosed with CTD-ILD were enrolled based on the inclusion and exclusion criteria. Among them, 34 patients progressed to PPF during treatment, and 160 patients did not progress. The variables obtained at lambda$1se in LASSO regression were ANCA associated vasculitis, lymphocytes, albumin, erythrocyte sedimentation rate, and serum ferritin. Multivariate COX regression analysis showed that the extent of fibrosis, serum ferritin, albumin, and age were independent risk factors for the progression of CTD-ILD to PPF (all P<0.05). A prediction model was established based on the results of the multivariate COX regression analysis. The area under the receiver operator characteristic curve at 6 months, 9 months, and 12 months was 0.989, 0.931, and 0.797, respectively, indicating that the model has good discrimination and sensitivity, and good predictive efficacy. The calibration curve showed a good overlap between predicted and actual values. Conclusions The extent of fibrosis, serum ferritin, albumin, and age are independent risk factors for the progression of CTD-ILD to PPF. The model established based on this and externally validated shows good predictive efficacy.
Objective To improve the awareness of acute exacerbation of idiopathic pulmonary fibrosis ( AEIPF) and discuss its clinical characteristics, diagnosis, treatment and outcome. Methods The clinical data of patients with AEIPF from June 2006 to June 2011 in 11 hospitals in Jiangsu were collected and analyzed. Resluts There were 18 males and 3 females in the AEIPF patients with mean age of ( 67.4 ± 8.1) years. The duration from IPF diagnosis was ( 7.4 ±8.2) months. The duration of acute symptom before admission was ( 7.0 ±5.3) days. The distribution pattern of new groud-glass opacity was peripheral in 3 patients,multifocal in 5 patients, and diffuse in13 patients. All patients were treated with corticosteroid pulse therapy. Nine patients survived and 12 patients died. The mortality rate was 57.1% . Conclusions AEIPF progresses quickly and the mortality rate is very high. Corticosteroid pulse therapy is the mainstay of therapy in AEIPF patients.
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.