Objective:To investigate the efficacy and safety of mepolizumab in the treatment of patients with eosinophilic granulomatosis with polyangiitis (EGPA).Methods:This was a non-randomized controlled trials study.Purposive sampling was used to collect data of 20 EGPA patients treated with mepolizumab at the First Affiliated Hospital of Guangzhou Medical University from August 2021 to December 2023.The study evaluated the differences in Birmingham Vasculitis Activity Score (BVAS), blood eosinophil count and percentage, forced expiratory volume in one second (FEV1) as a percentage of predicted value (FEV 1%pred), and oral corticosteroid (OCS) maintenance dose before and after treatment to assess the efficacy of mepolizumab and observe the safety of patients during treatment. Results:Among the 20 patients with EGPA, there were 17 females (85.0%), with an average age of 42.0 (30.8, 52.5)years.After 4 months of treatment, the total response rate was 85.0%.The blood eosinophil count decreased from 0.7 (0.5, 1.5)×10 9/L to 0.1 (0, 0.1)×10 9/L ( Z=3.88, P<0.001). The FEV 1%pred increased from 70.4% (49.7%, 85.2%) to 79.9% (72.0%, 101.5%)( Z=2.24, P=0.025). The fractional exhaled nitric oxide (FeNO) level decreased from 80.0 (35.0, 111.5) ppb to 51.6 (28.3, 65.4) ppb ( Z=2.54, P=0.011). The oral corticosteroid (OCS) dosage was reduced from 25.0 (16.3, 30.0) mg/d to 5.9 (5.0, 7.5) mg/d ( Z=3.83, P<0.001). After 12 months of treatment, 5 patients still show significant improvement inthe above clinical indicators compared to those before treatment.The clinical indicator changes before and after treatment between the standard dose group (300 mg/4 weeks) and the non-standard dose group (100 mg/4 weeks) were compared.The results showed no significant statistical difference in reducing BVAS scores, decreasing blood eosinophil counts, improving lung function indicators (FEV 1%pred), and reducing OCS dosage after 4 months of treatment ( P>0.05). Among the 20 patients, a total of 125 injections were administered, with only one instance of rash occurring post-injection of mepolizumab, which was tolerable. Conclusions:Mepolizumab has shown good efficacy in the treatment of EGPA, canreduce the maintenance dose of OCS andimprove lung function, and demonstrates good safety with no observed serious adverse events.Both the standard dose group and the non-standard dose group can effectively manage the symptoms and reduce the OCS usage in EGPA.
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive fibrotic lung disease with unclear etiology and pathogenesis.Currently, there is no effective treatment.In the past few decades, multiple clinical trials have been conducted to determine the safety and efficacy of pharmacological treatments for IPF patients.So far, only two drugs, pirfenidone and nintedanib, have been proven to effectively slow down the functional decline and disease progression of IPF, which were approved by the U. S.Food and Drug Administration (FDA) in 2014.In addition, the specific therapeutic effects and mechanisms of a series of anti-fibrotic drugs targeting the pathogenesis of IPF, including connective tissue growth factor, autotaxin, lysophosphatidic acids, and integrin αvβ6, require further investigation.This review summarizes the advances of pharmacological therapy for IPF and provides ideas and basis for the research and development of novel targeted drugs.
Objective:To explore the clinical application of laser ablation to benign granulomatous tracheal stenosis.Methods:It was an observational study involving 30 patients with benign granulomatous tracheal stenosis who were treated with laser ablation in the Department of Respiratory and Critical Care Medicine, Changzhou Second People's Hospital Affiliated to Nanjing Medical University from January 2019 to September 2022 using the non-random sampling method. Other interventional treatment measures like electric snares and argon gas knife were combined with laser ablation if necessary. The airway diameter, improvement of tracheal stenosis, shortness of breath score, dyspnea index, pulmonary function indicators, occurrence of adverse reactions, partial pressure of oxygen in the arterial blood (PaO 2), partial pressure of carbon dioxide in the arterial blood (PaCO 2) and arterial oxygen saturation (SaO 2) were recorded. Bronchoscopy or chest CT was regularly re-examined to analyze restenosis. Patients were followed up and the quality of life was assessed using the Karnofsky Performance Scale (KPS). Results:Among the 30 patients, 22 cases had granulation tissue hyperplasia after tracheostomy, 4 cases had granulation tissue hyperplasia after tracheal intubation, and 4 cases had granulation tissue hyperplasia after tracheal stent placement. All patients were treated with laser ablation under a direct vision of the bronchoscope. A total of 43 laser ablations were performed on 30 patients. One week after laser ablation treatment, the airway diameter ([2.63±1.61] mm vs [8.96±2.25] mm, t=13.55), degree of tracheal stenosis ([80.63±11.96] vs [31.87±9.56], t=20.22), and shortness of breath score ([3.53±0.57] points vs [1.13±0.35] points, t=19.48), dyspnea index (Grade 0: 0[0.0%] vs 18[60.0%], Grade Ⅰ: 0[0.0%] vs 4 [13.3%], Grade Ⅱ: 0[0.0%] vs 7[23.3%], Grade Ⅲ: 4 [13.3%] vs 1[0.3%], Grade Ⅳ: 10[33.3%] vs 0[0.0%], Grade V: 16[53.3%] vs 0[0.0%], Z=46.32), lung function indicators (forced expiratory volume in 1 second: [1.37±0.13] L vs [1.76±0.15] L, t=12.64; forced vital capacity: [2.31±0.15] L vs [2.70±0.15] L, t=11.55), blood gas analysis indicators (PaO 2: [84.57±6.03] mmHg vs [94.57±4.07] mmHg, t=8.14; PaCO 2: [59.50±4.03] mmHg vs [42.30±2.77] mmHg, t=18.57; SaO 2: [82.63±4.41] mmHg vs [93.60±1.57] mmHg, t=12.64; 1 mmHg=0.133 kPa) and KPS score (<50 points: 17[56.7%] vs 5[16.7%]; 50-80 points: 12[40.0%] vs 16[53.3%]; >80 points: 1[3.3%] vs 9[30.0%], Z=3.64) were all significantly improved compared with those before treatment (all P<0.05). After 5-month follow-up on stenotic patients with enlarged airways, 13 cases developed restenosis after airway enlargement. Among all patients, 1 case developed tracheomalacia after surgery, and no serious adverse events were reported. After 5 months of the follow-up, effective rate was 90.0% (27/30). Conclusions:The short-term efficacy of laser ablation in patients with benign granulomatous tracheal stenosis is significant, but the long-term efficacy needs further evaluation.
Objective:To study the regulatory effect of circular RNAs (circRNAs) circ_0003998 on promoting the epithelial-mesenchymal transition (EMT) of lung adenocarcinoma cells and the underlying mechanism.Methods:It was an experimental study.The lung epithelial cell line BEAS-2B and lung adenocarcinoma cell lines A549, H1299, H1395, H1975 were cultured, and the expression levels of circ_0003998 and miR-218 in each cell lines were detected.A549 cells were transfected with small interfering RNA(siRNA) negative control (si-NC), circ_0003998 siRNA (si-circ_0003998), si-circ_0003998+ miR-NC inhibitor, si-circ_0003998+ miR-218 inhibitor, miR-NC or miR-218.Cell migration was detected by Transwell assay.The expression levels of circ_0003998, Bmi-1 mRNA, E-cadherin mRNA, N-cadherin mRNA and miR-218 were detected by fluorescent quantitative polymerase chain reaction.The protein levels of Bmi-1, E-cadherin and N-cadherin were detected by Western blotting.Dual-luciferase reporter assay was performed to detect the targeting relationship between miR-218 and circ_0003998, and that between miR-218 and Bmi-1.Rescue experiments were performed to verify that the role of silenced miR-218 in reversing the regulatory effect of si-circ_0003998.Results:Circ_0003998 was upregulated in lung adenocarcinoma cell lines than that of lung epithelial cell lines, and miR-218 was downregulated (all P<0.05), with the most significant changes in A549 cells.Knockdown of circ_0003998 downregulated circ_0003998 ( t=15.21), and protein and mRNA levels of N-cadherin ( t=7.82 and 9.76, respectively) and Bmi-1 ( t=9.93 and 12.02, respectively), reduced the number of migratory cells ( t=15.53), and upregulated miR-218 ( t=11.51) and the protein and mRNA levels of E-cadherin ( t=7.28 and 10.00, respectively) (all P<0.001). The luciferase activity of wild-type circ_0003998 plasmid and wild-type Bmi-1 plasmid in cells overexpressing miR-218 was significantly lower than those transfected with miR-NC ( t=13.61 and 14.68, respectively, all P<0.001). Compared with those transfected with si-circ_0003998+ miR-NC inhibitor, A549 cells transfected with si-circ_0003998+ miR-218 inhibitor presented significantly higher number of migratory cells, protein and mRNA levels of N-cadherin and Bmi-1, but lower expression level of miR-218 and protein and mRNA levels of E-cadherin (all P<0.05). Conclusions:Circ_0003998 promotes EMT of lung adenocarcinoma cells through the miR-218/Bmi-1 axis.
Objective:To explore the role of pirfenidone in alleviating pulmonary fibrosis in mice by downregulating JAK2, and the underlying mechanism.Methods:It was an experimental study.By simple random sampling, 30 female mice with 8-weeks old were randomly divided into three groups: control group, bleomycin group (intratracheal nebulized spray of bleomycin 5 mg/kg) and pirfenidone group (intratracheal nebulized spray of bleomycin 5 mg/kg plus intragastric administration of pirfenidone 20 mg/kg once every other day) with 10 mice in each group.The lung coefficient of each group was calculated.Pathological changes in lung tissues were observed by hematoxylin-eosin (HE) staining and Masson staining.Collagen contents were determined.Relevant proteins in lung tissues were detected by Western blotting.Immunofluorescence signal intensities of p-JAK2 and transforming growth factor-β 1 (TGF-β 1) and the localization of p-JAK2 in lung tissue sections were observed by fluorescence microscope.Serum levels of relevant proteins were detected by enzyme linked immunosorbent assay (ELISA). The mouse epithelial cell line MLE-12 was cultured and divided into three groups: blank group, model group (TGF-β 1 10 μg/L) and intervention group (TGF-β 1 10 μg/L plus pirfenidone 0.1, 0.5, 1 and 5 mmol/L). Western blotting was used to detect protein levels of relevant proteins in MLE-12 cells.Fluorescence microscopy was used to observe the immunofluorescence signal intensity and localization of p-JAK2 in MLE-12 cells.Western blotting was used to detect protein levels of relevant proteins in blank group, model group, LY2109761 groups (0.01, 0.05 and 0.5 μmol/L LY2109761 plus TGF-β 1 10 μg/L), blank group, model group, si-NC group (transfection of negative control plus TGF-β 1 10 μg/L) and si-JAK2 group (transfection of si-JAK2 plus TGF-β 1 10 μg/L). Results:The lung coefficient of mice in bleomycin group was significantly higher than that of control group ([1.16±0.17]% vs [0.78±0.07]%, P<0.001), which was significantly lower in the pirfenidone group than that of bleomycin group ([1.02±0.07]% vs [1.16±0.17]%, P<0.05). The collagen content of lung tissues in bleomycin group was significantly higher than that of control group ([24.85±1.83] vs [8.84±1.44], P<0.001), and which was significantly lower in pirfenidone group than that of bleomycin group ([9.42±3.07] vs [24.85±1.83], P<0.01). The expression levels of JAK2, p-JAK2, transforming growth factor-β receptor 2 (TGF-βR2) and TGF-β 1 in bleomycin group were significantly higher than those of control group ([0.37±0.02] vs [0.15±0.01], [0.38±0.01] vs [0.20±0.01], [0.88±0.03] vs [0.14±0.01], and [0.18±0.01] vs [0.09±0.01], respectively, all P<0.001), which were significantly lower in pirfenidone group than those of bleomycin group ([0.29±0.02] vs [0.37±0.02], [0.28±0.01] vs [0.38±0.01], [0.71±0.02] vs [0.88±0.03], and [0.09±0.06] vs [0.18±0.01], respectively, all P<0.01). The immunofluorescence signal intensities of p-JAK2 and TGF-β 1 in bleomycin group were significantly higher than those of control group, which were significantly lower in pirfenidone group than those of bleomycin group (all P<0.01). p-JAK2 was mostly located in nucleus.The expression levels of serum pulmonary surfactant protein-A, Krebs Von den Lungen-6 and TGF-β 1 in bleomycin group were significantly higher than those of control group, while the expression levels of pulmonary surfactant protein-A, pulmonary surfactant protein-D, Krebs Von den Lungen-6 and TGF-β 1 in pirfenidone group were significantly lower than those of bleomycin group (all P<0.05). The expression levels of JAK2, p-JAK2 and TGF-βR2 in the model group were significantly higher than those of blank group, while those in the intervention group with different concentrations were significantly lower than those of model group (all P<0.05). With the prolongation of culture time, the immunofluorescence signal of p-JAK2 in MLE-12 cells of model group gradually increased, which was mainly localized in the nucleus and cytoplasm.The expression levels of JAK2, TGF-βR1 and TGF-βR2 in the model group were significantly higher than those of blank group (all P<0.01), which, in the LY2109761 groups with different concentrations were significantly lower than those of model group (all P<0.05). The expression levels of JAK2, TGF-βR2 and TGF-β 1 in model group and si-NC group were significantly higher than those of blank group, which, in si-JAK2 group were significantly lower than those of si-NC group (all P<0.05). Conclusions:Pirfenidone alleviates pulmonary fibrosis in mice by downregulating JAK2 through inhibiting the JAK2/STAT3 signaling pathway and inhibiting the nuclear translocation of p-JAK2.
Interstitial lung disease (ILD) is a group of heterogeneous disorder characterized by inflammatory response and/or fibrosis in the lung interstitium with clear or unclear causes, which further causes damaged lung structure and dysfunction.It is featured by a complicated pathogenesis, atypical clinical manifestations, and various changes in imaging and pathological findings.Clinical diagnosis and treatment of ILD require a multidisciplinary team.A multidisciplinary discussion (MDD) increases the accuracy of ILD diagnosis and treatment, which has been recommended by international and domestic guidelines.However, a standard guideline of MDD for ILD is scant.Since 2018, a regular MDD for ILD cases has been performed once a week in China-Japan Friendship Hospital.We perform a multi-center MDD on ILD cases online to demonstrate the diagnostic and therapeutic process of ILD cases by multidisciplinary team.Based on our experiences, we obtain the support by Chinese Thoracic Society, Chinese Medical Association and Chinese Association of Chest Physicians.We organize the formulation of " Chinese Expert Consensus on Multidisciplinary Discussion of Interstitial Lung Disease", aiming to standardize clinical management of ILD-MDD and enhance diagnosis and treatment level of ILD.
Objective:To analyze the differentially expressed serum α-1, 6 fucosyltransferase (FUT8) in benign and malignant pulmonary ground-glass nodules (GGNs), and to explore its significance and feasibility as a potential biomarker in the diagnosis and treatment of pulmonary GGNs.Methods:Observational study.A total of 98 patients diagnosed with pulmonary GGNs by high-resolution computed tomography (HRCT)and hospitalized in the Department of Thoracic Surgery, the First Affiliated Hospital of Dalian Medical University from September 2020 to February 2021 were recruited in the experimental group using the non-random sampling method.They were allocated to the benign pulmonary GGN group (18 cases) and malignant pulmonary GGN group (80 cases) according to postoperative pathological results.At the same time, 22 healthy volunteers without the detection of pulmonary GGNs by HRCT were included in the control group.Enzyme-linked immunosorbent assay (ELISA) and Western blot were used to detect serum FUT8 levels in the control group, benign and malignant pulmonary GGN group.The correlation of serum FUT8 with carcinoembryonic antigen (CEA), squamous cell carcinoma antigen (SCC-Ag), cyto-keratin 19 fragment antigen 21-1 (CYFRA 21-1) and neuron specific enolase (NSE) were identified.The receiver operating characteristic (ROC) curves were depicted to assess the diagnostic efficacy of serum FUT8 on malignant pulmonary GGNs.Results:There was a significant difference in the mean serum FUT8 level in malignant pulmonary GGN group (199.82[184.27, 213.85] μg/L), benign pulmonary GGN group (139.63[132.32, 199.48] μg/L) and control group (173.48[120.40, 206.01] μg/L) measured by ELISA ( H=19.044, P<0.05). The serum FUT8 level in the malignant pulmonary GGN group was significantly higher than that in benign pulmonary GGN group and control group (both P<0.05). There was a significant difference in the mean protein level of FUT8 in malignant pulmonary GGN group (16 638.47±1 822.89), benign pulmonary GGN group (2 053.24±113.36) and control group (3 619.83±726.40) measured by Western blot ( F=49.78, P<0.001). The protein level of FUT8 in the malignant pulmonary GGN group was significantly higher than that in benign pulmonary GGN group and control group (both P<0.05). CEA, SCC-Ag, CYFRA21-1 and NSE levels were not correlated with serum FUT8 level (all P>0.05). The area under the curve (AUC) of serum FUT8 in diagnosing malignant pulmonary GGNs was 0.744 (95% CI: 0.637-0.852, P<0.01), with the Youden index, optimal cut-off, sensitivity and specificity of 0.525, 148.30 μg/L, 98.75% and 52.50%, respectively. Conclusions:Serum FUT8 level increases in patients with malignant pulmonary GGNs, which can be used as one of the biomarkers to assist the diagnosis of malignant pulmonary GGNs.
Objective:To explore the potential mechanisms of the Qibai Pingfei Capsule on immune cells and target genes in idiopathic pulmonary fibrosis (IPF) patients by analyzing single cell RNA sequencing (scRNA-seq) data from lung tissues of IPF patients, combined with the network pharmacology of Qibai Pingfei Capsule.Methods:The scRNA-seq data of lung tissues in IPF patients (IPF group) and normal individuals (control group) were first downloaded from the Gene Expression Omnibus (GEO). Twenty samples were randomly selected from each group using the random number table method to explore the immune cell population and its proportion changes in IPF.Differential gene analysis was conducted on each immune cell population, and genes with P<0.05 and |log Fold Changes (logFC)|>0.6 were identified as differentially expressed genes.Gene Ontology (GO)-biological process enrichment analysis on differentially expressed genes was performed to explore the potential molecular functions and mechanisms involved in the progression of IPF.The primary active molecules and target genes of Qibai Pingfei Capsule were obtained based on the Traditional Chinese Medicine Systems Pharmacology Database and Analysis Platform (TCMSP), and GO and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analysis were performed on target genes.The " active molecules-target genes-immune cells" network was constructed by mapping the target genes of Qibai Pingfei Capsule to differentially expressed genes of IPF immune cells.The GO and KEGG enrichment analysis were conducted to explore the potential mechanism of Qibai Pingfei Capsule in the treatment of IPF.Protein-protein interaction (PPI) analysis was conducted by the Search Tool for the Retrieval of Interacting Genes (STRING) database and Cytoscape software to understand the interaction relationship of target genes in the " active molecules-target genes-immune cells" network and to identify key target genes. Results:The scRNA-seq analysis revealed a total of 18 immune cell populations in IPF lung tissues, including B cells, T cells, and congenital lymphocytes.Compared with the control group, the proportion of conventional dendritic cells 2, mast cells, plasmacytoid dendritic cells and regulatory T cells increased significantly in the IPF group (all P<0.05). A total of 534 differentially expressed genes were obtained from these 18 immune cell populations, which were involved in multiple immune related biological processes such as lymphocyte activation, antigen processing and presentation, and leukocyte migration and chemotaxis.Qibai Pingfei Capsule contained 18 active molecules and 239 target genes, among which nine active molecules, such as bifendate, quercetin, and calycosin, targeted 17 immune cell populations except for plasmacytoid dendritic cells and 42 differentially expressed genes, including interleukin-1B (IL-1B), vascular endothelial growth factor-A (VEGFA) and fos proto-oncogene (FOS). These targeted differentially expressed genes were mainly enriched in reactive oxygen species or oxidative stress related pathways, as well as immune related signaling pathways, including interleukin-17 (IL-17), mitogen-activated protein kinases (MAPK) and tumor necrosis factor (TNF) signaling pathways. Conclusions:Qibai Pingfei Capsule may target multiple immune cells and regulate the immune inflammatory response of IPF patients through reactive oxygen species or oxidative stress related pathways, as well as immune related signaling pathways such as IL-17, MAPK and TNF.
Objective:To identify the risk factors for systemic sclerosis (SSc) combined with interstitial lung disease (ILD).Methods:It was an observational study involving 66 SSc patients who were admitted to the Affiliated Hospital of Xuzhou Medical University for treatment from June 2013 to November 2022 using a non-random sampling method.Based on the high resolution CT findings of the lungs, the patients were divided into two groups: SSc-ILD group (35 cases) and SSc group (31 cases). General information (gender, age, body mass index) and laboratory parameters (white blood cell [WBC] count, neutrophil count, hemoglobin, platelet [PLT] count, albumin, total bilirubin, cystatin C [CysC], total cholesterol, triglycerides, fibrinogen, erythrocyte sedimentation rate, C-reactive protein) were compared between the two groups.Multivariable logistic regression analysis was used to evaluate the independent risk factors for SSc combined with ILD, and receiver operating characteristic (ROC) curve analysis was performed to assess the predictive value of independent risk factors for the development of ILD in SSc patients.Results:Patients in the SSc-ILD group were significantly older than those in the SSc group ([51.69±12.80] years vs [45.32±12.11] years, t=2.07, P=0.043). WBC count, neutrophil count, PLT count, and CysC were all significantly higher in the SSc-ILD group compared to those in the SSc group (5.80[4.90, 7.55]×10 9/L vs 4.70[3.85, 6.50]×10 9/L, 4.05[2.66, 4.66]×10 9/L vs 2.86[2.32, 3.92]×10 9/L, [236.46±76.15]×10 9/L vs [171.06±59.79]×10 9/L, [996.29±236.01] μg/L vs [870.00±153.93] μg/L, respectively; all P<0.05). PLT count elevation and CysC elevation were identified as independent risk factors for SSc combined with ILD ( OR=1.018, 95% CI: 1.007-1.030, P=0.002; OR=1.005, 95% CI: 1.001-1.009, P=0.025). PLT count and CysC had predictive value for the development of ILD in SSc patients, with an area under the curve of 0.728 (95% CI: 0.607-0.848, P=0.002) and 0.661 (95% CI: 0.530-0.791, P=0.025), respectively.The optimal cutoff value for PLT count was 198.50×10 9/L, with a sensitivity of 68.6% and specificity of 71.0%.The optimal cutoff value for CysC was 1 025.00 μg/L, with a sensitivity of 40.0% and specificity of 83.9%. Conclusions:Elevation of PLT count and CysC are independent risk factors for SSc combined with ILD.
This paper reports a case of anti-melanoma differentiation-associated gene 5 (MDA-5) antibody positive dermatomyositis combined with interstitial lung disease.In the early stage, the patient has respiratory tract symptom as the first symptom, and presents repeated pleural effusion.Symptoms of connective tissue diseases like rash, muscle pain, and weakness are not reported.Chest computed tomography (CT) shows patchy high-density shadows in the lower lobes of both lungs.After repeated anti-infection and anti-tuberculosis treatment, the patient presents progressed disease conditions, atypical rashes on the face and elbow ulceration.The dermatomyositis-related interstitial lung disease is finally diagnosed by myositis antibody spectrum analysis and biopsy and pathological examination of muscle tissues.After anti-inflammatory, immunosuppressive and anti-fibrosis treatment, the patient′s condition improves.Through this case report and literature review, this article aims to improve the diagnosis and treatment of anti-MDA-5 antibody positive dermatomyositis complicated with interstitial lung disease.
Coexistence of emphysema and pulmonary fibrosis used to be termed as combined pulmonary fibrosis and emphysema (CPFE). Although there have been many studies on CPFE, its definition and clinical management have been rarely analyzed.In 2022, the " Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Asociacion Latinoamericana de Torax (ALAT) Research Statement" proposes the definition, characteristics, pathophysiological mechanisms, comprehensive management and research priorities of CPFE.CPFE is also recommended as a syndrome.This statement proposes the research definition and classification criteria of CPFE, and also recommends the thorough description of radiological and pathological patterns in the research on CPFE.This article interprets the statement on the definition of CPFE as a syndrome, comprehensive management of patients, and future research priorities.
In recent clinical trials, fibrotic interstitial lung disease (ILD) that shares the similar physiopathologic mechanism and behavior with those of idiopathic pulmonary fibrosis (IPF) have been uniformly termed as progressive fibrosing ILD (PF-ILD). However, there is no consensus on the definition and diagnostic criteria of PF-ILD." Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline (2022 Edition)" unifies the process of ILD with progressive exacerbation of fibrosis other than idiopathic pulmonary fibrosis, and newly termed as progressive pulmonary fibrosis (PPF). They also clarify the diagnostic criteria, and anti-fibrosis treatment of PPF.This article thoroughly interprets this guideline for PPF.
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease of unknown causes, ultimately leading to hypoxemia, respiratory failure, and even death.The incidence of IPF has been increasing in recent years, and its prognosis is poor.IPF is currently a challenging disease, the diagnosis and treatment of which have been highly concerned.Four years later, an international multidisciplinary expert committee has reviewed the diagnosis and treatment of IPF from the 2018 version, and once again updated the guidelines for the diagnosis and treatment of IPF.This review provides a detailed interpretation of the diagnosis and treatment of IPF in the " Idiopathic Pulmonary Fibrosis (an Update) and Progressive Pulmonary Fibrosis in Adults: An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline (2022 Edition)" .
Interstitial lung disease (ILD) is a group of restrictive lung diseases characterized by injury and inflammation of pulmonary interstitium, including the bronchial wall, blood vessels and connective tissue around alveoli.It contains more than 200 kinds of diseases, and their common pathological features are inflammation and fibrosis.The factors affecting the quality of life of ILD patients can be classified into intrapulmonary and extrapulmonary.The former mainly includes the impairment of respiratory function caused by pulmonary inflammation and fibrosis, and the latter includes extrapulmonary symptoms, exercise ability, behaviors (such as smoking), social isolation, economic burden, psychological status, etc.In addition to the treatment of intrapulmonary factors (anti-inflammatory, antitussive, anti-fibrosis, etc.), it is also necessary to consider the intervention of extrapulmonary factors.Pulmonary rehabilitation is a safe, effective and economical comprehensive intervention, which may alleviate dyspnea, fatigue, anxiety and depression, and improve the activity ability and the quality of life of ILD patients.This review discusses the benefits of pulmonary rehabilitation for ILD patients, introduces the comprehensive management of pulmonary rehabilitation technology in ILD patients, and the difficulties in the implementation of pulmonary rehabilitation.
Nodular panniculitis is a non-suppurative, autoimmune disease that originates in fat lobules, the etiology of which is not clear.It lacks typical clinical symptoms, and the imaging findings of lung involvement are variable and non-specific.Imaging presentations of nodular panniculitis are similar to those of hematogenous disseminated pulmonary tuberculosis and sarcoidosis, leading to the difficulties in differential diagnosis.Skin biopsy provides an opportunity for the definite diagnosis of nodular panniculitis.However, it is challenging to diagnose nodular panniculitis, especially the rare cases of nodular panniculitis with lung involvement.This paper reports the diagnosis and treatment of a patient with nodular panniculitis complicated with airway central pulmonary interstitial fibrosis, and reviews the relevant literatures, aiming to improve the understanding of the disease.
Pulmonary fibrosis (PF) is a persistent, progressive and diffuse lung disease that mainly affects the lung interstitium.Its aggravation is irreversible, and thus, PF seriously endangers human life and health.Although a considerable advancement has been made on the pathogenesis and treatment of PF, lung transplantation is the only effective therapeutic strategy.It is urgent to seek for more effective treatment methods.Exosomes are tiny, nanoscale extracellular vesicles secreted by almost all types of cells, which are crucial for intercellular communication, immunological control, inflammation, and cellular phenotypic modification.They also play a significant role in PF.This review aims to clarify the role of exosomes in the pathogenesis of PF and to describe the diagnostic and therapeutic targets of various exosome miRNAs in PF, thus providing new diagnostic and prognostic biomarkers for PF and its novel therapeutic strategies.
Idiopathic pulmonary fibrosis (IPF) is a kind of chronic, progressive, fibrosis interstitial pneumonia, which is mainly manifested as dry cough and progressive dyspnea that eventually lead to lung dysfunction or even death.High resolution computed tomography (HRCT) used to be an important diagnostic tool for IPF.In recent years, with the in-depth understanding of the disease and the progress of science and technology, other diagnostic methods for IPF have emerged.This article reviews the diagnostic methods for IPF, thus enhancing the diagnostic rate of IPF, providing early treatment and improving the prognosis.
Emphysema is a common fibrotic interstitial lung disease.The concurrence of both emphysema and fibrotic interstitial lung disease is designated as combined pulmonary fibrosis and emphysema (CPFE). Due to the lack of the definition and diagnostic criteria of CPFE globally, the " Syndrome of Combined Pulmonary Fibrosis and Emphysema: An Official American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/Asociacion Latinoamericana de Torax (ALAT) Research Statement" proposes a statement in 2022 to thoroughly describe CPFE.This article interprets the clinical manifestations, imaging and pathological features and relevant terms of CPFE in this statement, aiming to improve the clinical diagnosis and management of CPFE.
In this paper, one case of bronchial glomus tumor was confirmed by histopathology and immunohistochemical staining through bronchoscopic biopsy.Key word " bronchial glomus tumor (in Chinese) " was used to search the related articles for literature review in CNKI, Wanfang Database, and Weipu.com.The patient, an elderly female, was admitted to the Second Affiliated Hospital of Dalian Medical University with cough, expectoration, and dyspnea as the chief complaints.Chest CT showed two nodules in the bronchial lumen, which were pathologically consistent with glomus tumor.In the database, 42 articles were retrieved, including a total of 70 cases, aged 18-84 years, with an average age of 48 years, and the male to female ratio was 1.2∶1.The common symptoms were chest tightness, shortness of breath, dyspnea (48.6%), cough (68.6%), hemoptysis (50%), and wheezing (11.4%). The maximum diameter of the tumor was 1.0-7.0 cm.The lesions mainly involved airway 65.7% and the left and right main bronchus 28.6%.Bronchial glomus tumor is rare, with no specific clinical and imaging manifestations.Surgical or bronchoscopic intervention can be used for treatment.
Chronic obstructive pulmonary disease (COPD) is a common respiratory disease with many comorbidities, which can significantly reduce life quality and increase mortality and medical costs in COPD patients.As a common chest imaging examination, chest computed tomography (CT) can evaluate the airway condition, emphysema degree, and the risk of acute exacerbation of COPD patients.Meanwhile, it can also evaluate COPD comorbidities in early stages, so as to provide an objective basis for individual treatment.This article reviews the role of chest CT in the evaluation of COPD disease and comorbidities.
