BACKGROUND Idiopathic pulmonary fibrosis(IPF)is classified under fibrotic interstitial pneumonia,characterized by a chronic and progressive course.The predominant clinical features of IPF include dyspnea and pulmonary...BACKGROUND Idiopathic pulmonary fibrosis(IPF)is classified under fibrotic interstitial pneumonia,characterized by a chronic and progressive course.The predominant clinical features of IPF include dyspnea and pulmonary dysfunction.AIM To assess the effects of pirfenidone in the early treatment of IPF on lung function in patients.METHODS A retrospective analysis was performed on 113 patients with IPF who were treated in our hospital from November 2017 to January 2023.These patients were divided into two groups:control group(n=53)and observation group(n=60).In the control group,patients received routine therapy in combination with methylprednisolone tablets,while those in the observation group received routine therapy together with pirfenidone.After applying these distinct treatment approaches to the two groups,we assessed several parameters,including the overall effectiveness of clinical therapy,the occurrence of adverse reactions(e.g.,nausea,vomiting,and anorexia),symptom severity scores,pulmonary function index levels,inflammatory marker levels,and the 6-min walk distance before and after treatment in both groups.RESULTS The observation group exhibited significantly higher rates than the control group after therapy,with a clear distinction(P<0.05).After treatment,the observation group experienced significantly fewer adverse reactions than the control group,with a noticeable difference(P<0.05).When analyzing the symptom severity scores between the two groups of patients after treatment,the observation group had significantly lower scores than the control group,with a distinct difference(P<0.05).When comparing the pulmonary function index levels between the two groups of patients after therapy,the observation group displayed significantly higher levels than the control group,with a noticeable difference(P<0.05).Evaluating the inflammatory marker data(C-reactive protein,interleukin-2[IL-2],and IL-8)between the two groups of patients after therapy,the observation group exhibited significantly lower levels than the control group,with significant disparities(P<0.05).Comparison of the 6-min walking distance data between the two groups of patients after treatment showed that the observation group achieved significantly greater distances than the control group,with a marked difference(P<0.05).CONCLUSION Prompt initiation of pirfenidone treatment in individuals diagnosed with IPF can enhance pulmonary function,elevate inflammatory factor levels,and increase the distance covered in the 6-min walk test.This intervention is conducive to effectively decreasing the occurrence of adverse reactions in patients.展开更多
Objective:To evaluate the effect of trigonelline on bleomycin-induced idiopathic pulmonary fibrosis(IPF)and to explore its underlying mechanisms using network pharmacology.Methods:IPF was induced in C57BL/6 mice by a ...Objective:To evaluate the effect of trigonelline on bleomycin-induced idiopathic pulmonary fibrosis(IPF)and to explore its underlying mechanisms using network pharmacology.Methods:IPF was induced in C57BL/6 mice by a single intratracheal instillation of bleomycin(5 mg/kg).Trigonelline was administered at doses of 25,50,and 100 mg/kg/day orally from the 2nd day post-bleomycin induction up to the 14th day.In IPF-induced mice,lung coefficient,immune cell infiltration in bronchoalveolar lavage fluid,and oxidative stress were measured.Histological alterations in lung tissues were also assessed.Moreover,network pharmacology approach was conducted to reveal molecular interactions of bleomycin and trigonelline with targets of IPF.Results:Trigonelline treatment reduced bleomycin-induced oxidative stress and immune cell infiltration,and mitigated physiological changes in the lung tissues of mice.Moreover,trigonelline alleviated bleomycin-induced histological alterations in lung tissues.Network pharmacology analysis showed that bleomycin and trigonelline interacted with IPF targets,such as NFKB1,HDAC2,HIF1A,and TLR4.Conclusions:The interaction of trigonelline with key IPF targets and its ameliorative effects on lung damage and oxidative stress highlight its potential in treating IPF.It may be considered an antifibrotic agent for further clinical development.展开更多
This editorial comments on the study by Lei et al investigating the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis(IPF)published.This study evaluates t...This editorial comments on the study by Lei et al investigating the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis(IPF)published.This study evaluates the efficacy of early treatment with pirfenidone on lung function in patients with IPF.The early and advanced stages of IPF are defined,highlighting the drug's benefits.While prior research indicates pirfenidone's effectiveness in advanced IPF,this study focuses on its advantages in early stages.The study emphasizes the importance of computed tomography imaging alongside biochemical data and lung function tests for a comprehensive analysis of symptom relief.Results show that early intervention with pirfenidone significantly reduces disease progression and preserves lung function,underscoring its potential as a critical treatment strategy in early IPF.展开更多
In this editorial,we comment on the article by Lei et al,with a specific focus on the timing of the initiation of the antifibrotic agent pirfenidone(PFD)in the management of idiopathic pulmonary fibrosis(IPF)and its i...In this editorial,we comment on the article by Lei et al,with a specific focus on the timing of the initiation of the antifibrotic agent pirfenidone(PFD)in the management of idiopathic pulmonary fibrosis(IPF)and its impact on lung function of IPF patients.PFD is an antifibrotic agent that is widely used in the management of IPF in both early and advanced stages.It inhibits various pathways and has antifibrotic,anti-inflammatory,and antioxidant properties.Despite dosage lowering,PFD slowed IPF progression and maintained functional capacity.The 6-min walk distance test indicated that patients tolerated adverse events well,and PFD significantly reduced the incidence of progression episodes and death.Even when a single disease-progression event occurred,continuing PFD treatment had benefits.展开更多
BACKGROUND Idiopathic pulmonary fibrosis(IPF)is a progressive interstitial lung disease with a high mortality rate.On this basis,exploring potential therapeutic targets to meet the unmet needs of IPF patients is impor...BACKGROUND Idiopathic pulmonary fibrosis(IPF)is a progressive interstitial lung disease with a high mortality rate.On this basis,exploring potential therapeutic targets to meet the unmet needs of IPF patients is important.AIM To explore novel hub genes for IPF therapy.METHODS Here,we used public datasets to identify differentially expressed genes between IPF patients and healthy donors.Potential targets were considered based on multiple bioinformatics analyses,especially the correlation between hub genes and carbon monoxide diffusing capacity of carbon monoxide,forced vital capacity,and patient survival rate.The mRNA levels of the hub genes were determined through quantitative real-time polymerase chain reaction.RESULTS We found that TDO2 was upregulated in IPF patients and predicted poor prognosis.Surprisingly,single-cell RNA sequencing data analysis revealed significant enrichment of TDO2 in alveolar fibroblasts,indicating that TDO2 may participate in the regulation of proliferation and survival.Therefore,we verified the upregulated expression of TDO2 in an experimental mouse model of transforming growth factor-β(TGF-β)-induced pulmonary fibrosis.Furthermore,the results showed that a TDO2 inhibitor effectively suppressed TGF-β-induced fibroblast activation.These findings suggest that TDO2 may be a potential target for IPF treatment.Based on transcription factors-microRNA prediction and scRNA-seq analysis,elevated TDO2 promoted the IPF proliferation of fibroblasts and may be involved in the P53 pathway and aggravate ageing and persistent pulmonary fibrosis.CONCLUSION We provided new target genes prediction and proposed blocking TGF-βproduction as a potential treatment for IPF.展开更多
BACKGROUND Rationale:No other treatment besides lung transplant is effective for idiopathic pulmonary fibrosis(IPF).Patients with IPF have poor prognosis,which may eventually lead to death.Patient concerns:Two female ...BACKGROUND Rationale:No other treatment besides lung transplant is effective for idiopathic pulmonary fibrosis(IPF).Patients with IPF have poor prognosis,which may eventually lead to death.Patient concerns:Two female patients were diagnosed with IPF.In our recent follow-up,both these patients maintained a good quality of life.CASE SUMMARY Diagnosis:Both patients had dry cough and progressive dyspnea.Interventions:The first patient was treated with prednisone,and the second patient was treated with prednisone and tripterygium glycosides.However,the symptoms did not improve and fibrosis was not controlled.Thus,the Feibi recipe was used.Outcomes:No deterioration was observed after the treatment,and the dry cough and its effect were ameliorated.Furthermore,they are still alive and the quality of their lives has improved.CONCLUSION These two cases suggest that the Feibi recipe and other traditional Chinese medicine therapies could be beneficial for IPF treatment.展开更多
Idiopathic pulmonary interstitial fibrosis is one of the respiratory refractory diseases,and the incidence rate is on the rise.At present,the effect of western medicine is not ideal and the side effects are obvious,wh...Idiopathic pulmonary interstitial fibrosis is one of the respiratory refractory diseases,and the incidence rate is on the rise.At present,the effect of western medicine is not ideal and the side effects are obvious,while the traditional Chinese medicine shows good curative effect on the disease.This paper makes a summary on the traditional Chinese medicine theory in treating idiopathic pulmonary interstitial fibrosis in recent years.展开更多
Idiopathic pulmonary fibrosis(IPF),characterized by aggravated alveolar destruc-tion and fibrotic matrix deposition,tendentiously experiences the stage called acute exacerbation IPF(AE-IPF)and progresses to multiple o...Idiopathic pulmonary fibrosis(IPF),characterized by aggravated alveolar destruc-tion and fibrotic matrix deposition,tendentiously experiences the stage called acute exacerbation IPF(AE-IPF)and progresses to multiple organ damage,especially liver injury.Recent studies have found a variety of immune microenvironment disorders associated with elevated IPF risk and secondary organ injury,whereas current animal models induced with bleomycin(BLM)could not completely reflect the pathologi-cal manifestations of AE-IPF patients in clinic,and the exact underlying mechanisms are not yet fully explored.In the current study,we established an AE-IPF model by tracheal administration of a single dose of BLM and then repeated administrations of lipopolysaccharide in mice.This mouse model successfully recapitulated the clinical features of AE-IPF,including excessive intrapulmonary inflammation and fibrosis and extrapulmonary manifestations,as indicated by significant upregulation of Il6,Tnfa,Il1b,Tgfb,fibronectin,and Col1a1 in both lungs and liver and elevated serum aspartate transaminase and alanine transaminase levels.These effects might be attributed to the regulation of Th17 cells.By sharing this novel murine model,we expect to pro-vide an appropriate experimental platform to investigate the pathogenesis of AE-IPF coupled with liver injury and contribute to the discovery and development of targeted interventions.展开更多
Dry powder inhaler Liposomes were prepared to investigate the effectiveness of pulmonary delivery of Colchicine and Budesonide for Idiopathic Pulmonary fibrosis. Budesonide(BUD) and Colchicine(COL) liposomes were prep...Dry powder inhaler Liposomes were prepared to investigate the effectiveness of pulmonary delivery of Colchicine and Budesonide for Idiopathic Pulmonary fibrosis. Budesonide(BUD) and Colchicine(COL) liposomes were prepared by thin layer film hydration method(TFH) using 1,2-Dipalmitoyl-sn-glycero-3-phosphoglycerol sodium(DPPG), Hydrogenated Soyaphosphotidylcholine(HSPC), Soyaphosphatidylcholine(SPC), cholesterol(CHOL) and drug in different weight ratios. The optimum lipid composition for BUD(74.22 ± 0.97%) was DPPG:HSPC: CHOL(4:5:1) and for COL(50.94 ± 2.04%) was DPPG: SPC: CHOL(3:6:1). These compositions retained drug for a longer period of time so selected for further study. Liposomes were found to be spherical in shape with mean size below 100 nm. Liposomes lyophilized using Mannitol as carrier and cryoprotectant showed high entrapment efficiency(97.89-98.6%). The powder was dispersed through an Andersen cascade impactor to evaluate the performance of the aerosolized powder. It was found that prepared liposomal dry powder inhaler(DPIs) sustained the drug release up to 24 hours. Optimized Budesonide DPI Formulation B2(86.53 ± 1.9%), Colchicine DPI Formulation C2(90.54 ± 2.3 %) and BUD and COL DPI Combination M2(89.91 ± 1.8%, 91.23 ± 1.9%). Histopathological results, measurements of lung hydroxyproline content, Myeloperoxidase activity indicated that liposomal drypowder inhaler administration attenuates lung fibrosis induced by bleomycin. Long term stability studies indicated that lyophilised BUD and COL liposomes were stable for 6 months at(25 °C± 2 °C, 60% ± 5% RH) and refrigerated conditions(2-8 °C). These results supported that combination of budesonide and colchicine liposomal dry powder inhaler pulmonary drug delivery for treatment of idiopathic Pulmonary Fibrosis exhibits prolonged drug retention at targeted site and reduces the systemic exposure.展开更多
AIM： To investigate the cumulative development incidence and predictive factors for idiopathic pulmonary fibrosis in hepatitis C virus （HCV） positive patients. METHODS： We studied 6150 HCV infected patients who we...AIM： To investigate the cumulative development incidence and predictive factors for idiopathic pulmonary fibrosis in hepatitis C virus （HCV） positive patients. METHODS： We studied 6150 HCV infected patients who were between 40-70 years old （HCV-group）. Another 2050 patients with hepatitis B virus （HBV） were selected as control （HBV-group）. The mean observation period was 8.0 ± 5.9 years in HCV-group and 6.3 ± 5.5 years in HBV-group. The primary goal is the development of idiopathic pulmonary fibrosis （IPF） in both groups. The cumulative appearance rate of IPF and independent factors associated with the incidence rate of IPF were calculated using the Kaplan- Meier method and the Cox proportional hazard model. All of the studies were performed retrospectively by collecting and analyzing data from the patient records in our hospital. RESULTS： Fifteen patients in HCV-group developed IPF. On the other hand, none of the patients developed IPF in HBV-group. In HCV-group, the cumulative rates of IPF development were 0.3% at 10th year and 0.9% at 20th year. The IPF development rate in HCV-group was higher than that in HBV-group （P = 0.021）. The IPF development rate in patients with HCV or HBV was high with statistical significance in the following cases： （1） patients ≥ 55 years （P 〈 0.001）; （2） patients who had smoking index （package per day x year） of ≥20 （P = 0.002）; （3） patients with liver cirrhosis （P = 0.042）. CONCLUSION： Our results indicate that age, smoking and liver cirrhosis enhance the development of IPF in HCV positive patients.展开更多
OBJECTIVE Leukotriene B4(LTB4)biosynthesis and subsequently neutrophilic inflammation may provide a potential strategy for the treatment of acute lung injury(ALI)or idiopathic pulmonary fibrosis(IPF).To provide a pote...OBJECTIVE Leukotriene B4(LTB4)biosynthesis and subsequently neutrophilic inflammation may provide a potential strategy for the treatment of acute lung injury(ALI)or idiopathic pulmonary fibrosis(IPF).To provide a potential strategy for the treatment of ALI or IPF,we identified potent inhibitors of Leukotriene A4 hydrolase(LTA4H),a key enzyme in the biosynthesis of LTB4.METHODS In this study,we identified two known histone deacetylase(HDAC)inhibitors,suberanilohydroxamic acid(SAHA)and its analogue 4-(dimethylamino)-N-[7-(hydroxyamino)-7-oxoheptyl]benzamide(M344),as effective inhibitors of LTA4H using enzymatic assay,thermofluor assay,and X-ray crystallographic investigation.We next tested the effect of SAHA and M344 on endogenous LTB4 biosynthesis in neutrophils by ELISA and neutrophil migration by transwell migration assay.A murine experimental model of ALI was induced by lipopolysaccharide(LPS)inhalation.Histopathological analysis of lung tissue using H&E staining revealed the serious pulmonary damage caused by LPS treatment and the effect of the SAHA.We next examined m RNA and protein levels of pro-inflammatory cytokines in lung tissue and bronchoalveolar lavage fluid using q RT-PCR and ELISA to further investigate the underlying mechanisms of anti-inflammatory activities by SAHA.We also investigated the effects of SAHA and M344 on a murine experimental model of bleomycin(BLM)-induced IPF model.RESULTS The results of enzymatic assay and X-ray crystallography showed that both SAHA and M344 bind to LTA4H,significantly decrease LTB4 levels in neutrophil,and markedly diminish early neutrophilic inflammation in mouse models of ALI and IPF under a clinical safety dose.CONCLUSION Collectively,SAHA and M344 would provide promising agents with well-known clinical safety for potential treatment in patients with ALI and IPF via pharmacologically inhibiting LAT4H and blocking LTB4 biosynthesis.展开更多
Idiopathic pulmonary fibrosis(IPF)is a serious and fatal pulmonary inflammatory disease with an increasing incidenceworldwide.The drugs nintedanib and pirfenidone,are listed as conditionally recommended drugs in the“...Idiopathic pulmonary fibrosis(IPF)is a serious and fatal pulmonary inflammatory disease with an increasing incidenceworldwide.The drugs nintedanib and pirfenidone,are listed as conditionally recommended drugs in the“Evidence-Based Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis”.However,these two drugs have many adverse reactions in clinical application.Salvianolic acid B(Sal B),a water-soluble component of Salvia miltiorrhiza,could alleviate bleomycin-induced peroxidative stress damage,and prevent or delay the onset of IPF by regulating inflammatory factors and fibrotic cytokines during the disease’s progression.However,Sal B is poorly absorbed orally,and patient compliance is poor when administered intravenously.Therefore,there is an urgent need to find a new non-injection route of drug delivery.In this study,Sal B was used as model drug and l-leucine(LL)as excipient to prepare Sal B dry powder inhaler(Sal B-DPI)by spray drying method.Modern preparation evaluation methods were used to assess the quality of Sal B-DPI.Sal B-DPI is promising for the treatment of IPF,according to studies on pulmonary irritation evaluation,in vivo and in vitro pharmacodynamics,metabolomics,pharmacokinetics,and lung tissue distribution.展开更多
AIM: To research the natural course of idiopathic pulmonary fibrosis(IPF) with advanced non-small cell lung cancer(NSCLC) and the association between acuteMETHODS: From May 2007 through April 2011, 17 CT naive patient...AIM: To research the natural course of idiopathic pulmonary fibrosis(IPF) with advanced non-small cell lung cancer(NSCLC) and the association between acuteMETHODS: From May 2007 through April 2011, 17 CT naive patients with IPF and advanced NSCLC were enrolled. Patients were classified into best supportive care(BSC) group or CT group based on the patient's preference. Patients in the CT group received carboplatin(CBDCA)(AUC 5-6) plus paclitaxel(PTX)(175-200 mg/m2) on day 1 of each 21-d cycle as first-line therapy.RESULTS: All patients but one chose the CT group. In the CT group, the objective response rate was 38%. The most frequent toxicity ≥ grade 3 was neutropenia(88%). Two patients(12.5%) developed AE-IPF. The median progression-free survival, the median survival time and the 1-year survival rate were 4.1 mo, 8.7 mo and 35%, respectively. Second-line CT-related AE and CT-unrelated AE occurred in 2 and 3 patients(1: BSC group; 2: CT group), respectively. Seven(41%) of all patients developed AE-IPF throughout the clinical course, and 6 of 7 patients with AE-IPF died within one month.CONCLUSION: The incidence of AE-IPF was higher among IPF patients with advanced NSCLC than among those without NSCLC. CBDCA plus PTX regimen was tolerable and effective. However, AE-IPF has a fatal toxicity with or without CT in IPF patients with advanced NSCLC.展开更多
Objective:This study aimed to explore the lived experiences of the disease journey and patients'care needs with idiopathic pulmonary fibrosis(IPF).Methods:Face-to-face semi-structured interviews were conducted wit...Objective:This study aimed to explore the lived experiences of the disease journey and patients'care needs with idiopathic pulmonary fibrosis(IPF).Methods:Face-to-face semi-structured interviews were conducted with a purposive sampling of IPF patients admitted to the department of respiratory medicine in a tertiary hospital in Beijing.Interview data were analyzed using the thematic analysis method.In the end,16 patients were interviewed.Results:Four themes emerged from the qualitative data included the long and confusing journey to reach a diagnosis,living with the disease,understanding the disease and treatment and desire for continuity of care.A series of subthemes were also identified,including uncertainty of diagnosis,delaying the process,living with physical symptoms,living with emotional distress,loss of independence,uncertainty with the prognosis,questioning the cause of the disease,concerning the side effects of treatments,lacking continuity of care,and wanting a better quality ofhealthcare in community hospitals.Conclusions:Based on the findings,there is an urgent need to improve the care delivery to this vulnerable population in China.To meet their health needs,it is of paramount importance to develop effective education programs for health professionals and IPF patients and improve care models of healthcare systems,especially in remote areas,to enhance care continuity in the communities.展开更多
Idiopathic pulmonary fibrosis(IPF) is the most common and rapidly fatal among idiopathic interstitial pneumonias. Its clinical course is variable. A significant fraction of the population of patients display a slow di...Idiopathic pulmonary fibrosis(IPF) is the most common and rapidly fatal among idiopathic interstitial pneumonias. Its clinical course is variable. A significant fraction of the population of patients display a slow disease course and can remain stable for years, while other patients show a rapid progressive course and may die within few months from diagnosis. For these reasons estimating prognosis of IPF patients is extremely difficult and has important clinical repercussions on optimal patients management including patients referral for lung transplantation. Several studies have tried to address this key point in the course of the two last decades analyzing different clinical, functional, radiological and biological variables. The purpose of this review is to assess relevant studies published on this subject and to examine the variety of prognostic predictors proposed along with staging systems.展开更多
AIM: To characterize cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis(IPF) compared to healthy controls.METHODS: Fifty-six IPF patients were involved in the study. The control population cons...AIM: To characterize cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis(IPF) compared to healthy controls.METHODS: Fifty-six IPF patients were involved in the study. The control population consisted of 144 healthy volunteers without history of lung disease.All of the patients were diagnosed with IPF according to the American Thoracic Society/European Respiratory Society consensus statement. Polymorphisms in the interleukin(IL)-1, IL-1, IL-1R, IL-1RA, IL-2, IL-4, IL-6, IL-10, IL-12, tumour necrosis factor, interferon, transforming growth factor, IL-1, IL-2, IL-4 and IL-4RA genes were characterized by polymerase chain reaction with sequence-specific primers. Statistical analysis was performed using the Med Calc statistical software. A Bonferroni correction of significance at an alpha of 0.05 was used for multiple analyses. A corrected P value less than 0.0023(0.05/22) was considered significant. RESULTS: We found significant differences in the IL-4 promoter region polymorphisms between IPF patients and controls. Namely, polymorphisms of IL-4(-590) [computed tomography(CT) in 32 of 56 patients vs 27 of 144 controls; P < 0.0001] and IL-4(-33)(CT in 25 of 56 patients vs 27 of 144 controls; P = 0.0006) differed between both groups. With regard to haplotypes, we found differences in the frequencies for haplotype 1 of IL-4(-1098)(-590)(-33) between IPF and controls(TCC in 23 of 56, TTC in 10 of 56, and TTT in 21 of 56 patients vs TCC in 112 of 144, TTC in 0 of 144, and TTT in 32 of 144 controls; P < 0.0001). We did not find significant differences in gene polymorphism frequencies of other cytokines in the IPF group vs the controls. CONCLUSION: We hypothesize that IL-4 promoter polymorphisms could be involved in the pathogenesis of IPF, likely via enhancement of the Th2 cytokine milieu with exaggerated fibroproliferative healing.展开更多
Background: The mediastinum is composed primarily of fatty tissue that is surrounded by the lungs bilaterally. There is a lack in the published literature in studying changes in mediastinal fat in idiopathic pulmonary...Background: The mediastinum is composed primarily of fatty tissue that is surrounded by the lungs bilaterally. There is a lack in the published literature in studying changes in mediastinal fat in idiopathic pulmonary fibrosis (IPF). The purpose of this study was to determine whether the shape and dimensions of the anterior mediastinal fat in patients with IPF are different from that of a normal control group and to correlate the changes with disease severity. Design and Setting: This prospective case control study was done at the chest department of Assiut University Hospital on IPF patients from May 2010-September 2012. A questionnaire containing questions such as age, sex, clinical findings, high resolution computerized tomography (HRCT) score and pulmonary function tests (PFTs) was filled for patients and normal controls. Results: The IPF retrosternal AP dimension was significantly shorter (p = 0.03) and the transverse dimension was longer (p = 0.001) than that in the normal control group. The convex shape of the anterior mediastinum was predictive of IPF (p = 0.001), whereas concave shape was predictive of normal controls (p = 0.001). The change in anteroposterior diameter (AP) and transverse diameters showed significant correlation with the changes in FVC, DLCO and HRCT score. Conclusions: IPF patients had reduced retrosternal AP and increased transverse dimensions than those of the controls with convex shape of their anterior mediastinal fat. Changes in anterior mediastinal fat dimensions are correlated with lower FVC, DLCO and higher HRCT score. A larger sample size, better multicenteric study is needed to confirm the results of this study.展开更多
The purpose of this study was to investigate the correlation between idiopathic pulmonary fibrosis(IPF)and tumor markers to provide evidence for early screening of precancerous lesions.In our hospital from July 2017 t...The purpose of this study was to investigate the correlation between idiopathic pulmonary fibrosis(IPF)and tumor markers to provide evidence for early screening of precancerous lesions.In our hospital from July 2017 to May 2019,40 patients with IPF treatment were selected as the IPF group,and 40 patients with idiopathic pulmonary fibrosis with lung cancer(IPF-LC)were selected as the IPF-LC group.In the same period,40 healthy physical examinees were used as control group.Different types of patients in the IPF-LC group were divided into lung adenocarcinoma group,small cell lung cancer group and l squamous carcinoma group.The expression levels of tumor markers were detected in the three groups,the positive rates of tumor markers in IPF group,IPF-LC group and their subgroups were compared.The results showed that the levels of neuron specific enolase(NSE),antigen CYFRA211,carcinogenic antigen(CEA)and cancer antigen 125(CA125)in IPF and IPF-LC groups were significantly higher than those in control group(P<0.05).There was no significant difference in CEA and CYFRA211 between IPF-LC group and IPF group.The level of NSE in IPF-LC group was significantly higher than that in IPF group,while the level of CA125 was significantly lower than that in IPF group(P<0.0.5).The difference of positive rate of NES and CA125 in IPF-LC group and IFP group was statistically significant(P<0.05),there was no statistically significant difference in the positive rate of other indicators(P>0.05)The NSE positive rate of IPF group was significantly lower than that of IPF-LC group(P<0.05),the positive rates of other tumor markers were significantly lower than those of each subgroup of IPF-LC group(P<0.05).Therefore,tumor markers in IPF patients showed different degrees of increase,which is worthy of clinical attention.Among them,NSE can be used as an early screening indicator for IPF precancerous lesions.展开更多
Objective:To explore the therapeutic effect of Bufei Huaxian Decoction plus prednisone on patients with idiopathic pulmonary fibrosis. Methods:80 patients with idiopathic pulmonary fibrosis treated in our hospital fro...Objective:To explore the therapeutic effect of Bufei Huaxian Decoction plus prednisone on patients with idiopathic pulmonary fibrosis. Methods:80 patients with idiopathic pulmonary fibrosis treated in our hospital from January 2018 to January 2019 were randomly divided into prednisone group and combination group, 40 cases in each group. Prenisone group was treated with prednisone, while Bufei Huaxian Decoction plus prednisone was used in combination group. Maximum expiratory flow (PEF), forced expiratory volume (FEV1) and forced vital capacity (FVC) were measured. Hyaluronidase (HA), laminin (LN), procollagen type Ⅲ (PCIII), transforming growth factor-β1 (TGF-β1) and hypersensitive C-reactive eggs were detected by enzyme-linked immunosorbent assay (ELISA). The levels of hs-CRP, TNF-α and glutathione peroxidase (GSH-Px), superoxide dismutase (SOD), malondialdehyde (MDA) were measured by immunoturbidimetry. The levels of T lymphocyte subsets and therapeutic effects were compared between the two groups. Results:After treatment, the levels of PEF, FEV1 and FVC in the combined group were higher than those in the prednisone group (P<0.05). After treatment, the levels of HA, LN and PC-Ⅲ in the combined group were lower than those in the prednisone group (P<0.05). After treatment, the serum levels of TGF-β1, hs-CRP and TNF-α in the combined group were lower than those in the prednisone group (P<0.05). After treatment, the serum GSH-Px and SOD levels in the combined group were higher than those in the prednisone group, and MDA levels were lower than those in the prednisone group (P<0.05). After treatment, the levels of CD8 + in the combined group were lower than those in the prednisone group, and the levels of CD4 + and CD3 + were higher than those in the prednisone group (P<0.05). The total effective rate of combined group 95.00% was higher than that of prednisone group 80.00% (P<0.05). Conclusions:Bufei Huaxian Decoction plus prednisone can improve the pulmonary function of patients with idiopathic pulmonary fibrosis, reduce the severity of pulmonary fibrosis, and enhance the antioxidant capacity and immune function of patients. The therapeutic effect is remarkable.展开更多
Idiopathic pulmonary fibrosis(IPF)is a chronic,progressive,fibrotic interstitial lung disease.Current treatment options for IPF are limited.Radix Astragali(RA)and Radix Angelicae Sinensis(RAS),according to 5:1 ratio c...Idiopathic pulmonary fibrosis(IPF)is a chronic,progressive,fibrotic interstitial lung disease.Current treatment options for IPF are limited.Radix Astragali(RA)and Radix Angelicae Sinensis(RAS),according to 5:1 ratio composed of Danggui Buxue decoction(DGBXD),which have played an essential role in the treatment of IPF.This article reviewed the experimental research,clinical research,and progress of RA and RAS(DGBXD)treating IPF to provide a deeper scientific basis for the future experimental research and clinical research.展开更多
文摘BACKGROUND Idiopathic pulmonary fibrosis(IPF)is classified under fibrotic interstitial pneumonia,characterized by a chronic and progressive course.The predominant clinical features of IPF include dyspnea and pulmonary dysfunction.AIM To assess the effects of pirfenidone in the early treatment of IPF on lung function in patients.METHODS A retrospective analysis was performed on 113 patients with IPF who were treated in our hospital from November 2017 to January 2023.These patients were divided into two groups:control group(n=53)and observation group(n=60).In the control group,patients received routine therapy in combination with methylprednisolone tablets,while those in the observation group received routine therapy together with pirfenidone.After applying these distinct treatment approaches to the two groups,we assessed several parameters,including the overall effectiveness of clinical therapy,the occurrence of adverse reactions(e.g.,nausea,vomiting,and anorexia),symptom severity scores,pulmonary function index levels,inflammatory marker levels,and the 6-min walk distance before and after treatment in both groups.RESULTS The observation group exhibited significantly higher rates than the control group after therapy,with a clear distinction(P<0.05).After treatment,the observation group experienced significantly fewer adverse reactions than the control group,with a noticeable difference(P<0.05).When analyzing the symptom severity scores between the two groups of patients after treatment,the observation group had significantly lower scores than the control group,with a distinct difference(P<0.05).When comparing the pulmonary function index levels between the two groups of patients after therapy,the observation group displayed significantly higher levels than the control group,with a noticeable difference(P<0.05).Evaluating the inflammatory marker data(C-reactive protein,interleukin-2[IL-2],and IL-8)between the two groups of patients after therapy,the observation group exhibited significantly lower levels than the control group,with significant disparities(P<0.05).Comparison of the 6-min walking distance data between the two groups of patients after treatment showed that the observation group achieved significantly greater distances than the control group,with a marked difference(P<0.05).CONCLUSION Prompt initiation of pirfenidone treatment in individuals diagnosed with IPF can enhance pulmonary function,elevate inflammatory factor levels,and increase the distance covered in the 6-min walk test.This intervention is conducive to effectively decreasing the occurrence of adverse reactions in patients.
文摘Objective:To evaluate the effect of trigonelline on bleomycin-induced idiopathic pulmonary fibrosis(IPF)and to explore its underlying mechanisms using network pharmacology.Methods:IPF was induced in C57BL/6 mice by a single intratracheal instillation of bleomycin(5 mg/kg).Trigonelline was administered at doses of 25,50,and 100 mg/kg/day orally from the 2nd day post-bleomycin induction up to the 14th day.In IPF-induced mice,lung coefficient,immune cell infiltration in bronchoalveolar lavage fluid,and oxidative stress were measured.Histological alterations in lung tissues were also assessed.Moreover,network pharmacology approach was conducted to reveal molecular interactions of bleomycin and trigonelline with targets of IPF.Results:Trigonelline treatment reduced bleomycin-induced oxidative stress and immune cell infiltration,and mitigated physiological changes in the lung tissues of mice.Moreover,trigonelline alleviated bleomycin-induced histological alterations in lung tissues.Network pharmacology analysis showed that bleomycin and trigonelline interacted with IPF targets,such as NFKB1,HDAC2,HIF1A,and TLR4.Conclusions:The interaction of trigonelline with key IPF targets and its ameliorative effects on lung damage and oxidative stress highlight its potential in treating IPF.It may be considered an antifibrotic agent for further clinical development.
文摘This editorial comments on the study by Lei et al investigating the efficacy of early treatment with pirfenidone on the lung function of patients with idiopathic pulmonary fibrosis(IPF)published.This study evaluates the efficacy of early treatment with pirfenidone on lung function in patients with IPF.The early and advanced stages of IPF are defined,highlighting the drug's benefits.While prior research indicates pirfenidone's effectiveness in advanced IPF,this study focuses on its advantages in early stages.The study emphasizes the importance of computed tomography imaging alongside biochemical data and lung function tests for a comprehensive analysis of symptom relief.Results show that early intervention with pirfenidone significantly reduces disease progression and preserves lung function,underscoring its potential as a critical treatment strategy in early IPF.
文摘In this editorial,we comment on the article by Lei et al,with a specific focus on the timing of the initiation of the antifibrotic agent pirfenidone(PFD)in the management of idiopathic pulmonary fibrosis(IPF)and its impact on lung function of IPF patients.PFD is an antifibrotic agent that is widely used in the management of IPF in both early and advanced stages.It inhibits various pathways and has antifibrotic,anti-inflammatory,and antioxidant properties.Despite dosage lowering,PFD slowed IPF progression and maintained functional capacity.The 6-min walk distance test indicated that patients tolerated adverse events well,and PFD significantly reduced the incidence of progression episodes and death.Even when a single disease-progression event occurred,continuing PFD treatment had benefits.
文摘BACKGROUND Idiopathic pulmonary fibrosis(IPF)is a progressive interstitial lung disease with a high mortality rate.On this basis,exploring potential therapeutic targets to meet the unmet needs of IPF patients is important.AIM To explore novel hub genes for IPF therapy.METHODS Here,we used public datasets to identify differentially expressed genes between IPF patients and healthy donors.Potential targets were considered based on multiple bioinformatics analyses,especially the correlation between hub genes and carbon monoxide diffusing capacity of carbon monoxide,forced vital capacity,and patient survival rate.The mRNA levels of the hub genes were determined through quantitative real-time polymerase chain reaction.RESULTS We found that TDO2 was upregulated in IPF patients and predicted poor prognosis.Surprisingly,single-cell RNA sequencing data analysis revealed significant enrichment of TDO2 in alveolar fibroblasts,indicating that TDO2 may participate in the regulation of proliferation and survival.Therefore,we verified the upregulated expression of TDO2 in an experimental mouse model of transforming growth factor-β(TGF-β)-induced pulmonary fibrosis.Furthermore,the results showed that a TDO2 inhibitor effectively suppressed TGF-β-induced fibroblast activation.These findings suggest that TDO2 may be a potential target for IPF treatment.Based on transcription factors-microRNA prediction and scRNA-seq analysis,elevated TDO2 promoted the IPF proliferation of fibroblasts and may be involved in the P53 pathway and aggravate ageing and persistent pulmonary fibrosis.CONCLUSION We provided new target genes prediction and proposed blocking TGF-βproduction as a potential treatment for IPF.
基金Supported by Beijing Natural Science Foundation,No.7202118National Natural Science Foundation of China,No.81573970and Basic Scientific Research Foundation of Beijing University of Chinese Medicine,No.2021-JYB-XJSJJ-033.
文摘BACKGROUND Rationale:No other treatment besides lung transplant is effective for idiopathic pulmonary fibrosis(IPF).Patients with IPF have poor prognosis,which may eventually lead to death.Patient concerns:Two female patients were diagnosed with IPF.In our recent follow-up,both these patients maintained a good quality of life.CASE SUMMARY Diagnosis:Both patients had dry cough and progressive dyspnea.Interventions:The first patient was treated with prednisone,and the second patient was treated with prednisone and tripterygium glycosides.However,the symptoms did not improve and fibrosis was not controlled.Thus,the Feibi recipe was used.Outcomes:No deterioration was observed after the treatment,and the dry cough and its effect were ameliorated.Furthermore,they are still alive and the quality of their lives has improved.CONCLUSION These two cases suggest that the Feibi recipe and other traditional Chinese medicine therapies could be beneficial for IPF treatment.
基金Name:Clinical study of FeiXianTong Decoction improving activity tolerance in patients with idiopathic pulmonary fibrosis.Subject is from Beijing municipal commission of science and technology(number:Z131107002213053)
文摘Idiopathic pulmonary interstitial fibrosis is one of the respiratory refractory diseases,and the incidence rate is on the rise.At present,the effect of western medicine is not ideal and the side effects are obvious,while the traditional Chinese medicine shows good curative effect on the disease.This paper makes a summary on the traditional Chinese medicine theory in treating idiopathic pulmonary interstitial fibrosis in recent years.
基金supported by the Innovation Team and Talents Cultivation Program of the National Administration of Traditional Chinese Medicine(grant no.:ZYYCXTD-C-202006 to XG and Xiaojiaoyang Li)Beijing Municipal Science and Technology Commission(grant no.:7212174 to Xiaojiaoyang Li)+2 种基金National Natural Science Foundation of China(grant no.:82004045 to Xiaojiaoyang Li)Beijing Nova Program of Science and Technology(grant no.:Z191100001119088 to Xiaojiaoyang Li)the Young Elite Scientists Sponsorship Program by CACM(grant no.:2020-QNRC2-01 to Xiaojiaoyang Li).
文摘Idiopathic pulmonary fibrosis(IPF),characterized by aggravated alveolar destruc-tion and fibrotic matrix deposition,tendentiously experiences the stage called acute exacerbation IPF(AE-IPF)and progresses to multiple organ damage,especially liver injury.Recent studies have found a variety of immune microenvironment disorders associated with elevated IPF risk and secondary organ injury,whereas current animal models induced with bleomycin(BLM)could not completely reflect the pathologi-cal manifestations of AE-IPF patients in clinic,and the exact underlying mechanisms are not yet fully explored.In the current study,we established an AE-IPF model by tracheal administration of a single dose of BLM and then repeated administrations of lipopolysaccharide in mice.This mouse model successfully recapitulated the clinical features of AE-IPF,including excessive intrapulmonary inflammation and fibrosis and extrapulmonary manifestations,as indicated by significant upregulation of Il6,Tnfa,Il1b,Tgfb,fibronectin,and Col1a1 in both lungs and liver and elevated serum aspartate transaminase and alanine transaminase levels.These effects might be attributed to the regulation of Th17 cells.By sharing this novel murine model,we expect to pro-vide an appropriate experimental platform to investigate the pathogenesis of AE-IPF coupled with liver injury and contribute to the discovery and development of targeted interventions.
文摘Dry powder inhaler Liposomes were prepared to investigate the effectiveness of pulmonary delivery of Colchicine and Budesonide for Idiopathic Pulmonary fibrosis. Budesonide(BUD) and Colchicine(COL) liposomes were prepared by thin layer film hydration method(TFH) using 1,2-Dipalmitoyl-sn-glycero-3-phosphoglycerol sodium(DPPG), Hydrogenated Soyaphosphotidylcholine(HSPC), Soyaphosphatidylcholine(SPC), cholesterol(CHOL) and drug in different weight ratios. The optimum lipid composition for BUD(74.22 ± 0.97%) was DPPG:HSPC: CHOL(4:5:1) and for COL(50.94 ± 2.04%) was DPPG: SPC: CHOL(3:6:1). These compositions retained drug for a longer period of time so selected for further study. Liposomes were found to be spherical in shape with mean size below 100 nm. Liposomes lyophilized using Mannitol as carrier and cryoprotectant showed high entrapment efficiency(97.89-98.6%). The powder was dispersed through an Andersen cascade impactor to evaluate the performance of the aerosolized powder. It was found that prepared liposomal dry powder inhaler(DPIs) sustained the drug release up to 24 hours. Optimized Budesonide DPI Formulation B2(86.53 ± 1.9%), Colchicine DPI Formulation C2(90.54 ± 2.3 %) and BUD and COL DPI Combination M2(89.91 ± 1.8%, 91.23 ± 1.9%). Histopathological results, measurements of lung hydroxyproline content, Myeloperoxidase activity indicated that liposomal drypowder inhaler administration attenuates lung fibrosis induced by bleomycin. Long term stability studies indicated that lyophilised BUD and COL liposomes were stable for 6 months at(25 °C± 2 °C, 60% ± 5% RH) and refrigerated conditions(2-8 °C). These results supported that combination of budesonide and colchicine liposomal dry powder inhaler pulmonary drug delivery for treatment of idiopathic Pulmonary Fibrosis exhibits prolonged drug retention at targeted site and reduces the systemic exposure.
基金Grants-in-Aid from Okinaka Memorial Institute for Medical Research and the Japanese Ministry of Health, Labour and Welfare
文摘AIM： To investigate the cumulative development incidence and predictive factors for idiopathic pulmonary fibrosis in hepatitis C virus （HCV） positive patients. METHODS： We studied 6150 HCV infected patients who were between 40-70 years old （HCV-group）. Another 2050 patients with hepatitis B virus （HBV） were selected as control （HBV-group）. The mean observation period was 8.0 ± 5.9 years in HCV-group and 6.3 ± 5.5 years in HBV-group. The primary goal is the development of idiopathic pulmonary fibrosis （IPF） in both groups. The cumulative appearance rate of IPF and independent factors associated with the incidence rate of IPF were calculated using the Kaplan- Meier method and the Cox proportional hazard model. All of the studies were performed retrospectively by collecting and analyzing data from the patient records in our hospital. RESULTS： Fifteen patients in HCV-group developed IPF. On the other hand, none of the patients developed IPF in HBV-group. In HCV-group, the cumulative rates of IPF development were 0.3% at 10th year and 0.9% at 20th year. The IPF development rate in HCV-group was higher than that in HBV-group （P = 0.021）. The IPF development rate in patients with HCV or HBV was high with statistical significance in the following cases： （1） patients ≥ 55 years （P 〈 0.001）; （2） patients who had smoking index （package per day x year） of ≥20 （P = 0.002）; （3） patients with liver cirrhosis （P = 0.042）. CONCLUSION： Our results indicate that age, smoking and liver cirrhosis enhance the development of IPF in HCV positive patients.
基金supported by National Natural Science Foundation of China(81402482,91313303)
文摘OBJECTIVE Leukotriene B4(LTB4)biosynthesis and subsequently neutrophilic inflammation may provide a potential strategy for the treatment of acute lung injury(ALI)or idiopathic pulmonary fibrosis(IPF).To provide a potential strategy for the treatment of ALI or IPF,we identified potent inhibitors of Leukotriene A4 hydrolase(LTA4H),a key enzyme in the biosynthesis of LTB4.METHODS In this study,we identified two known histone deacetylase(HDAC)inhibitors,suberanilohydroxamic acid(SAHA)and its analogue 4-(dimethylamino)-N-[7-(hydroxyamino)-7-oxoheptyl]benzamide(M344),as effective inhibitors of LTA4H using enzymatic assay,thermofluor assay,and X-ray crystallographic investigation.We next tested the effect of SAHA and M344 on endogenous LTB4 biosynthesis in neutrophils by ELISA and neutrophil migration by transwell migration assay.A murine experimental model of ALI was induced by lipopolysaccharide(LPS)inhalation.Histopathological analysis of lung tissue using H&E staining revealed the serious pulmonary damage caused by LPS treatment and the effect of the SAHA.We next examined m RNA and protein levels of pro-inflammatory cytokines in lung tissue and bronchoalveolar lavage fluid using q RT-PCR and ELISA to further investigate the underlying mechanisms of anti-inflammatory activities by SAHA.We also investigated the effects of SAHA and M344 on a murine experimental model of bleomycin(BLM)-induced IPF model.RESULTS The results of enzymatic assay and X-ray crystallography showed that both SAHA and M344 bind to LTA4H,significantly decrease LTB4 levels in neutrophil,and markedly diminish early neutrophilic inflammation in mouse models of ALI and IPF under a clinical safety dose.CONCLUSION Collectively,SAHA and M344 would provide promising agents with well-known clinical safety for potential treatment in patients with ALI and IPF via pharmacologically inhibiting LAT4H and blocking LTB4 biosynthesis.
基金supported by Innovation Team and Talents Cultivation Program of National Administration of Traditional Chinese Medicine (No. ZYYCXTD-D-202002)Scientific Research Project of Tianjin Municipal Education Commission (No.2019KJ083)
文摘Idiopathic pulmonary fibrosis(IPF)is a serious and fatal pulmonary inflammatory disease with an increasing incidenceworldwide.The drugs nintedanib and pirfenidone,are listed as conditionally recommended drugs in the“Evidence-Based Guidelines for the Diagnosis and Treatment of Idiopathic Pulmonary Fibrosis”.However,these two drugs have many adverse reactions in clinical application.Salvianolic acid B(Sal B),a water-soluble component of Salvia miltiorrhiza,could alleviate bleomycin-induced peroxidative stress damage,and prevent or delay the onset of IPF by regulating inflammatory factors and fibrotic cytokines during the disease’s progression.However,Sal B is poorly absorbed orally,and patient compliance is poor when administered intravenously.Therefore,there is an urgent need to find a new non-injection route of drug delivery.In this study,Sal B was used as model drug and l-leucine(LL)as excipient to prepare Sal B dry powder inhaler(Sal B-DPI)by spray drying method.Modern preparation evaluation methods were used to assess the quality of Sal B-DPI.Sal B-DPI is promising for the treatment of IPF,according to studies on pulmonary irritation evaluation,in vivo and in vitro pharmacodynamics,metabolomics,pharmacokinetics,and lung tissue distribution.
基金Supported by The Clinical Research Support Center Kyush(http://www.cres-kyushu.or.jp/)
文摘AIM: To research the natural course of idiopathic pulmonary fibrosis(IPF) with advanced non-small cell lung cancer(NSCLC) and the association between acuteMETHODS: From May 2007 through April 2011, 17 CT naive patients with IPF and advanced NSCLC were enrolled. Patients were classified into best supportive care(BSC) group or CT group based on the patient's preference. Patients in the CT group received carboplatin(CBDCA)(AUC 5-6) plus paclitaxel(PTX)(175-200 mg/m2) on day 1 of each 21-d cycle as first-line therapy.RESULTS: All patients but one chose the CT group. In the CT group, the objective response rate was 38%. The most frequent toxicity ≥ grade 3 was neutropenia(88%). Two patients(12.5%) developed AE-IPF. The median progression-free survival, the median survival time and the 1-year survival rate were 4.1 mo, 8.7 mo and 35%, respectively. Second-line CT-related AE and CT-unrelated AE occurred in 2 and 3 patients(1: BSC group; 2: CT group), respectively. Seven(41%) of all patients developed AE-IPF throughout the clinical course, and 6 of 7 patients with AE-IPF died within one month.CONCLUSION: The incidence of AE-IPF was higher among IPF patients with advanced NSCLC than among those without NSCLC. CBDCA plus PTX regimen was tolerable and effective. However, AE-IPF has a fatal toxicity with or without CT in IPF patients with advanced NSCLC.
基金The study was supported by grants from Beijing Excellent Talents Training Programme(2018000021469G220)Beijing Hospitals Authority Youth Programme(QML20200309),Beijing,China.
文摘Objective:This study aimed to explore the lived experiences of the disease journey and patients'care needs with idiopathic pulmonary fibrosis(IPF).Methods:Face-to-face semi-structured interviews were conducted with a purposive sampling of IPF patients admitted to the department of respiratory medicine in a tertiary hospital in Beijing.Interview data were analyzed using the thematic analysis method.In the end,16 patients were interviewed.Results:Four themes emerged from the qualitative data included the long and confusing journey to reach a diagnosis,living with the disease,understanding the disease and treatment and desire for continuity of care.A series of subthemes were also identified,including uncertainty of diagnosis,delaying the process,living with physical symptoms,living with emotional distress,loss of independence,uncertainty with the prognosis,questioning the cause of the disease,concerning the side effects of treatments,lacking continuity of care,and wanting a better quality ofhealthcare in community hospitals.Conclusions:Based on the findings,there is an urgent need to improve the care delivery to this vulnerable population in China.To meet their health needs,it is of paramount importance to develop effective education programs for health professionals and IPF patients and improve care models of healthcare systems,especially in remote areas,to enhance care continuity in the communities.
文摘Idiopathic pulmonary fibrosis(IPF) is the most common and rapidly fatal among idiopathic interstitial pneumonias. Its clinical course is variable. A significant fraction of the population of patients display a slow disease course and can remain stable for years, while other patients show a rapid progressive course and may die within few months from diagnosis. For these reasons estimating prognosis of IPF patients is extremely difficult and has important clinical repercussions on optimal patients management including patients referral for lung transplantation. Several studies have tried to address this key point in the course of the two last decades analyzing different clinical, functional, radiological and biological variables. The purpose of this review is to assess relevant studies published on this subject and to examine the variety of prognostic predictors proposed along with staging systems.
基金Supported by Grants from the Internal Grant Agency of Ministry of Health of the Czech Republic:9131-3/2007,NS 10423-3/2009 and NT13433-4/2012
文摘AIM: To characterize cytokine gene polymorphisms in patients with idiopathic pulmonary fibrosis(IPF) compared to healthy controls.METHODS: Fifty-six IPF patients were involved in the study. The control population consisted of 144 healthy volunteers without history of lung disease.All of the patients were diagnosed with IPF according to the American Thoracic Society/European Respiratory Society consensus statement. Polymorphisms in the interleukin(IL)-1, IL-1, IL-1R, IL-1RA, IL-2, IL-4, IL-6, IL-10, IL-12, tumour necrosis factor, interferon, transforming growth factor, IL-1, IL-2, IL-4 and IL-4RA genes were characterized by polymerase chain reaction with sequence-specific primers. Statistical analysis was performed using the Med Calc statistical software. A Bonferroni correction of significance at an alpha of 0.05 was used for multiple analyses. A corrected P value less than 0.0023(0.05/22) was considered significant. RESULTS: We found significant differences in the IL-4 promoter region polymorphisms between IPF patients and controls. Namely, polymorphisms of IL-4(-590) [computed tomography(CT) in 32 of 56 patients vs 27 of 144 controls; P < 0.0001] and IL-4(-33)(CT in 25 of 56 patients vs 27 of 144 controls; P = 0.0006) differed between both groups. With regard to haplotypes, we found differences in the frequencies for haplotype 1 of IL-4(-1098)(-590)(-33) between IPF and controls(TCC in 23 of 56, TTC in 10 of 56, and TTT in 21 of 56 patients vs TCC in 112 of 144, TTC in 0 of 144, and TTT in 32 of 144 controls; P < 0.0001). We did not find significant differences in gene polymorphism frequencies of other cytokines in the IPF group vs the controls. CONCLUSION: We hypothesize that IL-4 promoter polymorphisms could be involved in the pathogenesis of IPF, likely via enhancement of the Th2 cytokine milieu with exaggerated fibroproliferative healing.
文摘Background: The mediastinum is composed primarily of fatty tissue that is surrounded by the lungs bilaterally. There is a lack in the published literature in studying changes in mediastinal fat in idiopathic pulmonary fibrosis (IPF). The purpose of this study was to determine whether the shape and dimensions of the anterior mediastinal fat in patients with IPF are different from that of a normal control group and to correlate the changes with disease severity. Design and Setting: This prospective case control study was done at the chest department of Assiut University Hospital on IPF patients from May 2010-September 2012. A questionnaire containing questions such as age, sex, clinical findings, high resolution computerized tomography (HRCT) score and pulmonary function tests (PFTs) was filled for patients and normal controls. Results: The IPF retrosternal AP dimension was significantly shorter (p = 0.03) and the transverse dimension was longer (p = 0.001) than that in the normal control group. The convex shape of the anterior mediastinum was predictive of IPF (p = 0.001), whereas concave shape was predictive of normal controls (p = 0.001). The change in anteroposterior diameter (AP) and transverse diameters showed significant correlation with the changes in FVC, DLCO and HRCT score. Conclusions: IPF patients had reduced retrosternal AP and increased transverse dimensions than those of the controls with convex shape of their anterior mediastinal fat. Changes in anterior mediastinal fat dimensions are correlated with lower FVC, DLCO and higher HRCT score. A larger sample size, better multicenteric study is needed to confirm the results of this study.
文摘The purpose of this study was to investigate the correlation between idiopathic pulmonary fibrosis(IPF)and tumor markers to provide evidence for early screening of precancerous lesions.In our hospital from July 2017 to May 2019,40 patients with IPF treatment were selected as the IPF group,and 40 patients with idiopathic pulmonary fibrosis with lung cancer(IPF-LC)were selected as the IPF-LC group.In the same period,40 healthy physical examinees were used as control group.Different types of patients in the IPF-LC group were divided into lung adenocarcinoma group,small cell lung cancer group and l squamous carcinoma group.The expression levels of tumor markers were detected in the three groups,the positive rates of tumor markers in IPF group,IPF-LC group and their subgroups were compared.The results showed that the levels of neuron specific enolase(NSE),antigen CYFRA211,carcinogenic antigen(CEA)and cancer antigen 125(CA125)in IPF and IPF-LC groups were significantly higher than those in control group(P<0.05).There was no significant difference in CEA and CYFRA211 between IPF-LC group and IPF group.The level of NSE in IPF-LC group was significantly higher than that in IPF group,while the level of CA125 was significantly lower than that in IPF group(P<0.0.5).The difference of positive rate of NES and CA125 in IPF-LC group and IFP group was statistically significant(P<0.05),there was no statistically significant difference in the positive rate of other indicators(P>0.05)The NSE positive rate of IPF group was significantly lower than that of IPF-LC group(P<0.05),the positive rates of other tumor markers were significantly lower than those of each subgroup of IPF-LC group(P<0.05).Therefore,tumor markers in IPF patients showed different degrees of increase,which is worthy of clinical attention.Among them,NSE can be used as an early screening indicator for IPF precancerous lesions.
基金Scientific research project of Guangdong traditional Chinese Medicine Bureau(No.20171246)“Three famous medical and health projects”in Shenzhen(No.SZSM201812063).
文摘Objective:To explore the therapeutic effect of Bufei Huaxian Decoction plus prednisone on patients with idiopathic pulmonary fibrosis. Methods:80 patients with idiopathic pulmonary fibrosis treated in our hospital from January 2018 to January 2019 were randomly divided into prednisone group and combination group, 40 cases in each group. Prenisone group was treated with prednisone, while Bufei Huaxian Decoction plus prednisone was used in combination group. Maximum expiratory flow (PEF), forced expiratory volume (FEV1) and forced vital capacity (FVC) were measured. Hyaluronidase (HA), laminin (LN), procollagen type Ⅲ (PCIII), transforming growth factor-β1 (TGF-β1) and hypersensitive C-reactive eggs were detected by enzyme-linked immunosorbent assay (ELISA). The levels of hs-CRP, TNF-α and glutathione peroxidase (GSH-Px), superoxide dismutase (SOD), malondialdehyde (MDA) were measured by immunoturbidimetry. The levels of T lymphocyte subsets and therapeutic effects were compared between the two groups. Results:After treatment, the levels of PEF, FEV1 and FVC in the combined group were higher than those in the prednisone group (P<0.05). After treatment, the levels of HA, LN and PC-Ⅲ in the combined group were lower than those in the prednisone group (P<0.05). After treatment, the serum levels of TGF-β1, hs-CRP and TNF-α in the combined group were lower than those in the prednisone group (P<0.05). After treatment, the serum GSH-Px and SOD levels in the combined group were higher than those in the prednisone group, and MDA levels were lower than those in the prednisone group (P<0.05). After treatment, the levels of CD8 + in the combined group were lower than those in the prednisone group, and the levels of CD4 + and CD3 + were higher than those in the prednisone group (P<0.05). The total effective rate of combined group 95.00% was higher than that of prednisone group 80.00% (P<0.05). Conclusions:Bufei Huaxian Decoction plus prednisone can improve the pulmonary function of patients with idiopathic pulmonary fibrosis, reduce the severity of pulmonary fibrosis, and enhance the antioxidant capacity and immune function of patients. The therapeutic effect is remarkable.
基金Jiangyin Hospital of Traditional Chinese Medicine(202014 to YF Zhang)Grants from the Wuxi Health Commission’s Scientific Research Project(M202154 to YF Zhang)。
文摘Idiopathic pulmonary fibrosis(IPF)is a chronic,progressive,fibrotic interstitial lung disease.Current treatment options for IPF are limited.Radix Astragali(RA)and Radix Angelicae Sinensis(RAS),according to 5:1 ratio composed of Danggui Buxue decoction(DGBXD),which have played an essential role in the treatment of IPF.This article reviewed the experimental research,clinical research,and progress of RA and RAS(DGBXD)treating IPF to provide a deeper scientific basis for the future experimental research and clinical research.
