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.
Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute and clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormality. In the past, AE-IPF was considered to be idiopathic, which was hard to be prevented and its prognosis was hard to be obviously improved; the latest researches have shown that AE-IPF can be triggered by known causes, including pulmonary infection, aspiration, etc. This review summarizes the etiology or risk factors, treatment and prevention of AE-IPF according to the latest researches.
Objective To explore the correlation and mechanism of ferroptosis with pulmonary fibrosis. Methods Pulmonary fibrosis tissue sequencing data were obtained from Gene Expression Omnibus and FerrDb databases from January 2019 to December 2023. Differentially expressed genes (DEGs) between the normal control group and the pulmonary fibrosis group were analyzed by bioinformatic method, and DEGs related to pulmonary iron addiction were extracted. The hub genes were screened by enrichment analysis, protein-protein interaction (PPI) analysis and random forest algorithm. The mouse model of pulmonary fibrosis was made for exercise intervention, and the expression of hub genes was verified by real-time quantitative reverse transcription polymerase chain reaction. Results A comparison of 103 patients with idiopathic pulmonary fibrosis and 103 normal lung tissues showed that 13 up-regulated genes and 7 down-regulated genes were identified as ferroptosis-related DEGs. PPI results showed that there was an interaction between these ferroptosis-related genes. The Kyoto Encyclopedia of Genes and Genomes pathway enrichment and Genome Ontology enrichment analysis showed that ferroptosis-related genes were involved in organic anion transport, hypoxia response, oxygen level reduction response, hypoxia-inducible factor-1 signaling pathway, renal cell carcinoma, and arachidonic acid metabolic signaling pathway. Genes identified by PPI analysis and random forest algorithm included CAV1, NOS2, GDF15, HNF4A, and CDKN2A. Real-time fluorescence quantitative polymerase chain reaction results of mouse fibrotic lung tissue showed that compared with the exercise group, the mRNA levels of NOS2, PTGS2 and GDF15 were up-regulated and the mRNA levels of CAV1 and CDKN2A were down-regulated in the bleomycin group (P<0.05); compared with the bleomycin group, the expression of CAV1 and CDKN2A increased and the expression of NOS2, PTGS2 and GDF15 decreased in the bleomycin + exercise group (P<0.05). Conclusions Bioinformatic analysis identifies 20 potential genes associating with ferroptosis in pulmonary fibrosis. CAV1, NOS2, GDF15, and CDKN2A influence the development of pulmonary fibrosis by modulating ferroptosis. Treadmill training can reduce ferroptosis in fibrotic tissues, thereby reducing lung inflammation.
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.
ObjectiveTo evaluate the clinical efficacy and safety of pirfenidone in Chinese patients with idiopathic pulmonary fibrosis (IPF). MethodsIn a multicenter,randomized,double-blind,comparative clinical trial,87 patients with IPF were randomly divided into two groups. Group A (43 patients) were treated with pirfenidone (1 200 mg per day) for 48 weeks,while Group B (44 patients) were treated with placebo. Clinical features were compared between two groups including efficacy indicators (pulmonary function,6MWT,and quality of life scores) and safety indicators (incidence of adverse events). ResultsForced vital capacity (FVC) was increased by (90±410)mL in Group A and decreased by (70±310)mL in Group B (P<0.05);In Group A,forced expiratory volume in 1 second was raised by (100±330)mL and (110±240)mL following 12 and 24 weeks after treatment,significantly different from group B (P<0.05). There were significant differences in 6MWT between two groups 36 and 48 weeks after treatment respectively(both P<0.05). Quality of life scores,including the St. George's score (excluding symptoms) and dyspnea score,were significantly higher in Group A than Group B (both P<0.05). There was no significant difference in the incidence of adverse events between Groups A and B (83.72% vs. 72.73%,P>0.05). ConclusionDomestic pirfenidone is clinically effective and safe for the treatment of IPF in Chinese patients.
【Abstract】 Objective To evaluate the relationship between multiple tumor biomarkers and idiopathic pulmonary fibrosis ( IPF) , and analyze the prognostic value of these biomarkers in IPF. Methods Clinical data of 43 confirmed IPF patients with no evidence of malignant disaeses, admitted in Peking Union Medical College Hospital between January 2000 and June 2010, were retrospectively analyzed. All IPF patients had detected serum alpha fetoprotein ( AFP) , cancer antigen 50 ( CA50) , cancer antigen 24-2( CA24-2) , carcinoembryonic antigen ( CEA) , carbohydrate antigen 19-9 ( CA19-9) , cancer antigen 125( CA125) , cancer antigen 15-3 ( CA15-3) , tissue polypeptide antigen ( TPA) , neuron specific enolase( NSE) , and cytokeratin-19-fragment ( Cyfra211) . Results The serum levels of CEA, CA19-9, CA125,CA15-3, and TPA were obviously higher than normal range, while the serum levels of AFP, CA50, CA24-2,NSE, and Cyfra211 were within normal range. Neither tumor biomarkers had correlation with 6-minute walk distance, FVC% pred, TLC% pred, DLCO/VA, PaO2 , PaO2 /FiO2 , P( A-a) O2 , BALF cell differentiation counting,or CD4 /CD8. The patients with increased CA19-9 level had shorter survival time than those with normal CA19-9 level ( P lt; 0. 05) . There was no significant difference in survival time between the patients with increased CEA/TPA levels and those with normal CEA/TPA levels( P gt;0. 05) , neither between the patients with glucocorticoid treatment and those with non-glucocorticoid treatment ( P gt; 0. 05) . Conclusions Multiple tumor biomarkers, especially CA19-9, increase in IPF patients. The degrees of those increases arenot associated with the severity of disease, but closely relate to prognosis, and may also indicate the progression. The increases of multiple tumor biomarkers may be a sign of poor prognosis of IPF with no evidence of malignant disaeses.
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.
ObjectiveTo investigate the key long non-coding RNAs (lncRNAs) and transcription factors (TFs) in idiopathic pulmonary fibrosis (IPF) by Bioinformatics analysis.MethodsBioinformatics analysis of three gene expression profiles from the Gene Expression Omnibus dataset (GSE2052, GSE44723, and GSE24206), including 42 IPF and 21 normal lung tissues, was performed in this study. Subsequently, differentially expressed genes (DEGs) were filtered, and key genes involved in signaling pathways and the DEG-associated protein-protein interaction network (PPI) were further analyzed. The filtered genes expression was determined by real-time quantitative polymerase chain reaction analysis.ResultsA total of 8483 aberrantly expressed genes were screened, and 29 overlapping genes were identified among these three datasets. A significant enrichment analysis of DEG-associated functions and pathways was further performed. A total of 18 modules were obtained from the DEG PPI network, and most of the modules were involved in polyubiquitination, Golgi vesicle transport, endocytosis and so on. The key genes were obtained through hypergeometric testing, and most of the corresponding genes were closely associated with ubiquitin-mediated proteolysis, the spliceosome, and the cell cycle. These differential expressed genes, such as lncMALAT1, E2F1 and YBX1, were detected in the peripheral blood of IPF patients when compared with those normal control subjects.ConclusionlncMALAT1, E2F1 and YBX1 might be possible regulators for the pathogenesis of idiopathic pulmonary fibrosis.
Patients with coronavirus disease 2019 may have systemic symptoms of varying degrees. These symptoms are related to inflammatory response, massive release of pro-inflammatory cytokines and cytokine storm. In recent years, programmed necrosis, as a controllable type of necrosis, is considered to be an important factor that mediates inflammation. Recent studies have shown that programmed necrosis is involved in the inflammatory response and pulmonary fibrosis of coronavirus disease 2019. This article mainly reviews the mechanism of programmed necrosis, its participation in the occurrence and development of coronavirus disease 2019, and the research progress of programmed necrosis inhibitors in the treatment of coronavirus disease 2019, aiming to provide a certain basis for the diagnosis and treatment of coronavirus disease 2019.
ObjectiveTo evaluate the efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis. MethodsPubMed, Cochrane Library, CNKI, CBM, Wanfang, and VIP databases were searched for randomized controlled trials of pirfenidone as interventions for the treatment of idiopathic pulmonary fibrosis. According to the Cochrane system evaluation method, the methodological quality of included studies was evaluated and the effective data were extracted. The meta-analysis was performed with RevMan 5.2 software. ResultsSix studies were included with 1727 patients in total. Compared with placebo groups, pirfenidon could improve the changing rate of vital capacity at the end of the treatment[WMD=0.06, 95% CI (0.01, 0.12), Z=2.48, P=0.01; heterogeneity inspection χ2=1.03, P=0.31]. Pirfenidon could not improve the changing rate of lowest SpO2 in 6-minute walking test[WMD=0.82, 95% CI (-1.35, 2.98), Z=0.74, P=0.46; heterogeneity inspection χ2=8.90, P=0.003] and could not reduce the mortality[RR=0.62, 95% CI (0.37, 1.03), Z=1.85, P=0.06; heterogeneity inspection χ2=3.05, P=0.55]. The incidences of photosensitivity, dizziness, nausea, abdominal discomfort, joint pain, fatigue in pirfenidone group were more frequent than those in placebo group. ConclusionsBecause of lack of enough eligible studies and defects in design and reporting data in the studies, this meta-analysis can not evaluate pirfenidone's long-term efficacy and safety. Hence, the existed clinical evidences can't support pirfenidone to be the treatment of IPF medication.