Translational respiratory medicine最新文献

{"title":"The epidermal growth factor receptor (EGRF) in lung cancer.","authors":"Enric Carcereny,&nbsp;Teresa Morán,&nbsp;Laia Capdevila,&nbsp;Sara Cros,&nbsp;Laia Vilà,&nbsp;Maria de Los Llanos Gil,&nbsp;Jordi Remón,&nbsp;Rafael Rosell","doi":"10.1186/s40247-015-0013-z","DOIUrl":"10.1186/s40247-015-0013-z","url":null,"abstract":"<p><p>In the last decade, important advances have been made in understanding of cancer biology, particularly non-small-cell lung cancer (NSCLC) with the discovery of oncogenic drivers of the disease. The epidermal growth factor receptor (EGFR) gene and its pathways was the first oncogenic driver discovered to be mutated and treatable in lung cancer. Treatment with EGFR tyrosine kinase inhibitors (TKIs) is the standard of care for molecularly selected EGFR-mutant patients, while its role in unselected lung cancer patients is nowadays controversial. This review will provide an overview of the EGFR pathway and options for its treatment of lung cancer. </p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"3 ","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2015-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40247-015-0013-z","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33162884","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Response of airway epithelial cells to double-stranded RNA in an allergic environment.","authors":"Cristan Herbert,&nbsp;Qing-Xiang Zeng,&nbsp;Ramesh Shanmugasundaram,&nbsp;Linda Garthwaite,&nbsp;Brian G Oliver,&nbsp;Rakesh K Kumar","doi":"10.1186/s40247-014-0011-6","DOIUrl":"https://doi.org/10.1186/s40247-014-0011-6","url":null,"abstract":"<p><strong>Background: </strong>Respiratory viral infections are the most common trigger of acute exacerbations in patients with allergic asthma. The anti-viral response of airway epithelial cells (AEC) may be impaired in asthmatics, while cytokines produced by AEC may drive the inflammatory response. We investigated whether AEC cultured in the presence of Th2 cytokines associated with an allergic environment exhibited altered responses to double-stranded RNA, a virus-like stimulus.</p><p><strong>Methods: </strong>We undertook preliminary studies using the MLE-12 cell line derived from mouse distal respiratory epithelial cells, then confirmed and extended our findings using low-passage human AEC. Cells were cultured in the absence or presence of the Th2 cytokines IL-4 and IL-13 for 48 hours, then stimulated with poly I:C for 4 hours. Expression of relevant anti-viral response and cytokine genes was assessed by quantitative real-time PCR. Secretion of cytokine proteins was assessed by immunoassay.</p><p><strong>Results: </strong>Following stimulation with poly I:C, MLE-12 cells pre-treated with Th2 cytokines exhibited significantly higher levels of expression of mRNA for the cytokine genes Cxcl10 and Cxcl11, as well as a trend towards increased expression of Cxcl9 and Il6. Expression of anti-viral response genes was mostly unchanged, although Stat1, Ifit1 and Ifitm3 were significantly increased in Th2 cytokine pre-treated cells. Human AEC pre-treated with IL-4 and IL-13, then stimulated with poly I:C, similarly exhibited significantly higher expression of IL8, CXCL9, CXCL10, CXCL11 and CCL5 genes. In parallel, there was significantly increased secretion of CXCL8 and CCL5, as well as a trend towards increased secretion of CXCL10 and IL-6. Again, expression of anti-viral response genes was not decreased. Rather, there was significantly enhanced expression of mRNA for type III interferons, RNA helicases and other interferon-stimulated genes.</p><p><strong>Conclusion: </strong>The Th2 cytokine environment appears to promote increased production of pro-inflammatory chemokines by AEC in response to double-stranded RNA, which could help explain the exaggerated inflammatory response to respiratory viral infection in allergic asthmatics. However, any impairment of anti-viral host defences in asthmatics appears unlikely to be a consequence of Th2 cytokine-induced downregulation of the expression of viral response genes by AEC.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 1","pages":"11"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40247-014-0011-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32704294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Serial pulmonary function tests to diagnose COPD in chronic heart failure.","authors":"Armine G Minasian,&nbsp;Frank Jj van den Elshout,&nbsp;Pn Richard Dekhuijzen,&nbsp;Petra Je Vos,&nbsp;Frank F Willems,&nbsp;Paul Jpc van den Bergh,&nbsp;Yvonne F Heijdra","doi":"10.1186/s40247-014-0012-5","DOIUrl":"https://doi.org/10.1186/s40247-014-0012-5","url":null,"abstract":"<p><strong>Background: </strong>It is unknown whether serial pulmonary function tests are necessary for the correct diagnosis of chronic obstructive pulmonary disease (COPD) in patients with stable non-congested chronic heart failure (CHF). The aim of this study was to determine the prevalence of COPD in outpatients with stable CHF without pulmonary congestion using initial as well as confirmatory spirometry three months after treatment for COPD.</p><p><strong>Methods: </strong>Spirometry was performed in 187 outpatients with stable CHF without pulmonary congestion on chest radiograph who had a left ventricular ejection fraction < 40% (mean age 69 ± 10 years, 78% men). COPD was defined according to the Global Initiative for Chronic Obstructive Lung Disease guidelines. The diagnosis of COPD was confirmed three months after treatment with tiotropium in newly diagnosed COPD patients.</p><p><strong>Results: </strong>Using a three month follow-up spirometry to confirm initial diagnosis of de novo COPD did not change COPD prevalence significantly: 32.6% initially versus 32.1% after three months of follow-up. Only 1 of 25 (4%) patients with newly diagnosed COPD was not reproducibly obstructed at follow-up. COPD was greatly under- (19%) and overdiagnosed (32%).</p><p><strong>Conclusions: </strong>Spirometry should be used under stable and euvolemic conditions to decrease the burden of undiagnosed or overdiagnosed COPD in patients with CHF. Under these conditions, a confirmatory spirometry is unnecessary, as it does not change a newly established diagnosis of COPD in the vast majority of patients with CHF.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov Identifier NCT01429376.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 1","pages":"12"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40247-014-0012-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32722161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Performance of standard procedures in detection of EGFR mutations in daily practice in advanced NSCLC patients selected according to the ESMO guideline: a large Caucasian cohort study.","authors":"Inge Hantson,&nbsp;Christophe Dooms,&nbsp;Eric Verbeken,&nbsp;Peter Vandenberghe,&nbsp;Liesbet Vliegen,&nbsp;Tania Roskams,&nbsp;Sara Vander Borght,&nbsp;Kris Nackaerts,&nbsp;Isabelle Wauters,&nbsp;Johan Vansteenkiste","doi":"10.1186/s40247-014-0009-0","DOIUrl":"https://doi.org/10.1186/s40247-014-0009-0","url":null,"abstract":"<p><strong>Background: </strong>ESMO consensus recommends EGFR mutation testing in never/former light smokers (<15 pack-years) or patients with non-squamous NSCLC. The aim of this work was to determine the frequency and clinical predictors of EGFR mutations, and the role of specimen sampling tests, in Caucasian standard practice setting.</p><p><strong>Methods: </strong>We screened 297 patients according to this consensus. Mutational analysis of EGFR was performed using the Therascreen EGFR RGQ PCR mutation kit. Clinical and pathological correlative data were collected.</p><p><strong>Results: </strong>An EGFR activating mutation was found in 32 patients (11%), twelve exon 19 deletions, two exon 18 and eighteen exon 21 point mutations. Most were in females, but half were in smokers. Negative TTF-1 staining had a very strong negative predictive value (all except one patient had TTF-1 positive adenocarcinoma). Both biopsies as well as cytology specimens (mainly EBUS-TBNA) did well: 24 mutations in 213 biopsy samples (11.2%) and 8 in 84 cytology samples (9.5%), respectively. The Therascreen acted as a sensitive test in all types of samples: 7 activating mutations were found in samples rated to have <5% of tumour cells, and there were only 4 test failures in the whole series.</p><p><strong>Conclusion: </strong>In this Caucasian standard practice NSCLC cohort, tested according to the ESMO consensus, activating EGFR mutation occurred in 11% of the patients. Half of these were in former/current smokers. With our sampling technique and use of the Therascreen kit, EBUS-TBNA cell blocks performed as good as biopsies.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 1","pages":"9"},"PeriodicalIF":0.0,"publicationDate":"2014-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40247-014-0009-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32704293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Expression of transcript factors SALL4 and OCT4 in a subset of non-small cell lung carcinomas (NSCLC).","authors":"Erika Rodriguez, Li Chen, Ming-Hui Ao, Susan Geddes, Ed Gabrielson, Frederic Askin, Hui Zhang, Qing Kay Li","doi":"10.1186/s40247-014-0010-7","DOIUrl":"10.1186/s40247-014-0010-7","url":null,"abstract":"<p><strong>Background: </strong>SALL4 and OCT4 are transcription factors and play essential roles in stem cell development and oncogenesis. However, the expression of these transcription factors has not been well studied in lung cancers. In this study, we evaluated the expression of SALL4 and OCT4 in non-small cell lung carcinomas (NSCLC) by immunochemistry. NSCLC tissue microarrays (TMAs) were constructed with a total of 77 primary lung adenocarcinomas (ADCs) and 90 primary lung squamous cell carcinomas (SqCCs). A mouse monoclonal anti-human SALL4 (1:400 dilution) and a polyclonal anti-human OCT4 (1:200 dilution) antibodies were used. Nuclear staining of SALL4 and OCT4 was scored semi-quantitatively using a three tiered scale. The expressions of SALL4 and OCT4 were correlated with the tumor differentiation, pathological stage, and patients' clinical information.</p><p><strong>Results: </strong>In primary ADCs, the stronger expression of SALL4 and OCT4 was 7.8% and 9.1%, respectively. The stronger expression of SALL4 was inversely correlated with tumor differentiations. In primary SqCCs, the stronger expressions of SALL4 and OCT4 were 16.7% and 0%, respectively. The expression of SALL4 is correlated with the expression of OCT4, but inversely correlated with the tumor stage in SqCCs.</p><p><strong>Conclusions: </strong>We found that both SALL4 and OCT4 were differentially expressed in a subset of primary ADC and SqCC. Our finding suggest that different stem cell markers may be expressed and/or play differential role in different subtypes of NSCLC. The potential role of SALL4 and OCT4 needs to be further investigated in NSCLC.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 1","pages":"10"},"PeriodicalIF":0.0,"publicationDate":"2014-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4201749/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32773938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Inhibition of β-catenin/p300 interaction proximalizes mouse embryonic lung epithelium.","authors":"Tomoyo Sasaki,&nbsp;Michael Kahn","doi":"10.1186/s40247-014-0008-1","DOIUrl":"https://doi.org/10.1186/s40247-014-0008-1","url":null,"abstract":"<p><strong>Background: </strong>Wnt/β-catenin signaling has been suggested to regulate proximal-distal determination of embryonic lung epithelium based upon genetically modified mouse models. The previously identified and characterized small molecule inhibitor IQ1 can pharmacologically decrease the interaction between β-catenin and its transcriptional coactivator p300, thereby enhancing the β-catenin/CBP interaction. Inhibition of the β-catenin/p300 interaction by IQ1 blocks the differentiation of embryonic stem cells and epicardial progenitor cells; however, whether differential coactivator usage by β-catenin plays a role in proximal-distal determination of lung epithelium is unknown.</p><p><strong>Methods: </strong>We examined the effects of inhibiting the β-catenin/p300 interaction with IQ1 on lung branching morphogenesis in mouse embryos in utero and mouse embryonic lung organ culture ex vivo. The phenotype of IQ1 treated lungs was analyzed by epithelial staining, histology, quantitative PCR and in situ hybridization.</p><p><strong>Results: </strong>Inhibition of the β-catenin/p300 interaction by IQ1 disrupted the distal branching of mouse lung epithelium both in utero and ex vivo. IQ1 proximalized lung epithelium with decreased expression of the genes Bmp4 and Fgf10, hallmarks of distal lung determination, and increased expression of the proximal genes Sox2 and Scgb1a1 (CC10) as shown by quantitative PCR and in situ hybridization. The disruption of branching was reversible ex vivo as branching was reinitiated after removal of IQ1 from the media.</p><p><strong>Conclusions: </strong>The results demonstrate that the β-catenin/p300 interaction plays a critical role in proximal-distal determination of the epithelium in mouse lung branching morphogenesis and β-catenin/p300 inhibition pharmacologically proximalizes lung epithelium.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 ","pages":"8"},"PeriodicalIF":0.0,"publicationDate":"2014-09-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/s40247-014-0008-1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32905590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Looking for the elusive lung stem cell niche.","authors":"Ena Ray Banerjee","doi":"10.1186/2213-0802-2-7","DOIUrl":"10.1186/2213-0802-2-7","url":null,"abstract":"<p><p>This discourse contains three perspectives on various aspects of Stem Cell Biology and tools available to study and translate into Regenerative Medicine. The lung incessantly faces onslaught of the environment, constantly undergoes oxidative stress, and is an important organ of detoxification. In degenerative diseases and inflammation, the lung undergoes irreversible remodeling that is difficult to therapeutically address and/or transplant a dying tissue. The other difficulty is to study its development and regenerative aspects to best address the aforementioned problems. This perspective therefore addresses- firstly, review of types of stem cells, their pathway of action and models in invertebrate organisms vis-a-vis microenvironment and its dynamics; secondly, stem cells in higher organisms and niche; and lastly data and inference on a novel approach to study stem cell destruction patterns in an injury model and information on putative lung stem cell niche. Stem cells are cryptic cells known to retain certain primitive characteristics making them akin to ancient cells of invertebrates, developmental stages in invertebrates and vertebrates and pliant cells of complex creatures like mammals that demonstrate stimulus-specific behavious, whether to clonally propagate or to remain well protected and hidden within specialized niches, or mobilize and differentiate into mature functionally operative cells to house-keep, repair injury or make new tissues. In lung fibrosis, alveolar epithelium degenerates progressively. In keeping with the goal of regenerative medicine, various models and assays to evaluate long and short term identity of stem cells and their niches is the subject of this perspective. We also report identification and characterization of functional lung stem cells to clarify how stem cell niches counteract this degenerative process. Inferences drawn from this injury model of lung degeneration using a short term assay by tracking side population cells and a long term assay tracking label retaining cells have been presented. </p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 ","pages":"7"},"PeriodicalIF":0.0,"publicationDate":"2014-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4406986/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"33141539","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Secretion properties, clearance, and therapy in airway disease.","authors":"Bruce K Rubin","doi":"10.1186/2213-0802-2-6","DOIUrl":"https://doi.org/10.1186/2213-0802-2-6","url":null,"abstract":"<p><p>Chronic airway diseases like cystic fibrosis, chronic bronchitis, asthma, diffuse panbronchiolitis, and bronchiectasis are all associated with chronic inflammation. The airway mucosa responds to infection and inflammation in part by surface mucous (goblet) cell and submucosal gland hyperplasia and hypertrophy with mucus hypersecretion. Products of inflammation including neutrophil derived DNA and filamentous actin, effete cells, bacteria, and cell debris all contribute to mucus purulence and, when this is expectorated it is called sputum. Mucus is usually cleared by ciliary movement, and sputum is cleared by cough. These airway diseases each are associated with the production of mucus and sputum with characteristic composition, polymer structure, and biophysical properties. These properties change with the progress of the disease making it possible to use sputum analysis to identify the potential cause and severity of airway diseases. This information has also been important for the development of effective mucoactive therapy to promote airway hygiene. </p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 ","pages":"6"},"PeriodicalIF":0.0,"publicationDate":"2014-03-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/2213-0802-2-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32905589","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidemiology of bronchial asthma and asthma control assessment in Henan Province, China.","authors":"Wenping Zhang, Xianliang Chen, Lijun Ma, Jizhen Wu, Limin Zhao, Hongyan Kuang, Taibo Huang, Jianjian Cheng, Luoxian Zhang, Yong Qi, Beibei Sun, Hongyan Niu","doi":"10.1186/2213-0802-2-5","DOIUrl":"10.1186/2213-0802-2-5","url":null,"abstract":"<p><strong>Background: </strong>Prevalence of bronchial asthma, asthma treatment assessment, and estimation of the control level among asthma patients in Henan Province, China are reported in this paper.</p><p><strong>Methods: </strong>We selected 10 among the 109 cities and districts in Henan province using a multistage stratified cluster random sampling method. A total of 500 households from each city and district were chosen. Approximately 20,000 residents from a total of 5,000 households were randomly selected to answer a questionnaire recommended by the China Asthma Alliance. Asthma patients were asked to answer a detailed questionnaire using the symptom-based guidelines to assess the levels of disease control.</p><p><strong>Results: </strong>The overall prevalence of asthma was 0.73% ± 0.12%. Urban and rural residents had asthma prevalence rates of 1.1% ± 0.23% (88/7,924) and 0.48% ± 0.12% (57/11,792), respectively. Among the asthma patients, only 33.8% (52) received regular medication, 25% (13) used oral glucocorticoids, and 71.1% (37) used oral theophylline. The classified control levels of patients were as follows: 33.1% controlled, 49.7% partially controlled, and 17.2% uncontrolled. A total of 38.5% and 27.5% of regularly and irregularly treated asthma patients reached controlled level, respectively. The two groups significantly differed in asthma control level.</p><p><strong>Conclusion: </strong>Asthma prevalence is low in Henan Province, China. Urban residents have higher prevalence of asthma than rural residents do. Patients with asthma receive insufficient medication, resulting in suboptimal asthma control. Improvement in diagnosis and treatment of asthma patients is urgently needed.</p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 ","pages":"5"},"PeriodicalIF":0.0,"publicationDate":"2014-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4215820/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32905588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnosis and management of interstitial lung disease.","authors":"Keith C Meyer","doi":"10.1186/2213-0802-2-4","DOIUrl":"https://doi.org/10.1186/2213-0802-2-4","url":null,"abstract":"<p><p>The complex tasks of making a confident diagnosis of a specific form of interstitial lung disease (ILD) and formulating a patient-centered, personalized management plan in an attempt to achieve remission or stabilization of the disease process can pose formidable challenges to clinicians. When patients are evaluated for suspected ILD, an accurate diagnosis of the specific form of ILD that a patient has developed must be made to provide the patient with useful prognostic information and to formulate an appropriate management plan that can relieve symptoms and restore or significantly improve quality of life. A well-performed patient history and physical examination provides invaluable information that can be combined with appropriate laboratory testing, imaging, and, if needed, tissue biopsy to reach a confident ILD diagnosis, and high-resolution computed tomography (HRCT) of the thorax is usually a key component of the diagnostic evaluation. If treatment is indicated, many forms of ILD can respond significantly to immunosuppressive anti-inflammatory therapies. However, ILD accompanied by extensive fibrosis may be difficult to treat, and the identification of an effective pharmacologic therapy for idiopathic pulmonary fibrosis (IPF) has remained elusive despite the completion of many phase 3 clinical trials over the past decade. Nonetheless, patients with IPF or advanced forms of non-IPF ILD can benefit significantly from detection and treatment of various co-morbid conditions that are often found in patients (especially the elderly patient), and supportive care (oxygen therapy, pulmonary rehabilitation) can have a beneficial impact on quality of life and symptom palliation. Finally, lung transplantation is an option for patients with progressive, advanced disease that does not respond to other therapies, but only a relatively small subset of patients with end-stage ILD are able to meet wait listing requirements and eventually undergo successful lung transplantation. </p>","PeriodicalId":90074,"journal":{"name":"Translational respiratory medicine","volume":"2 ","pages":"4"},"PeriodicalIF":0.0,"publicationDate":"2014-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1186/2213-0802-2-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"32905587","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}