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Search Results: 1 - 10 of 6583 matches for " Idiopathic Pulmonary Fibrosis "
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Anterior Mediastinal Fat Changes in Idiopathic Pulmonary Fibrosis: A Preliminary Study  [PDF]
Wafaa Ali Hassan, Eman Abo-Elhamd
Open Journal of Respiratory Diseases (OJRD) , 2014, DOI: 10.4236/ojrd.2014.41003
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.
Increased survivin expression contributes to apoptosis-resistance in IPF fibroblasts  [PDF]
Thomas H. Sisson, Toby M. Maher, Iyabode O. Ajayi, Jessie E. King, Peter D.R. Higgins, Adam J. Booth, Rommel L. Sagana, Steven K. Huang, Eric S. White, Bethany B. Moore, Jeffrey C. Horowitz
Advances in Bioscience and Biotechnology (ABB) , 2012, DOI: 10.4236/abb.2012.326085
Abstract: Fibroblasts perform critical functions during the normal host response to tissue injury, but the inappropriate accumulation and persistent activation of these cells results in the development of tissue fibrosis. The mechanisms accounting for the aberrant accumulation of fibroblasts during fibrotic repair are poorly understood, although evidence supports a role for fibroblast resistance to apoptosis as a contributing factor. We have shown that TGF-β1 and endothelin-1 (ET-1), soluble mediators implicated in fibrogenesis, promote fibroblast resistance to apoptosis. Moreover, we recently found that ET-1 induced apoptosis resistance in normal lung fibroblasts through the upregulation of survivin, a member of the Inhibitor of Apoptosis (IAP) protein family. In the current study, we sought to determine the role of survivin in the apoptosis resistance of primary fibroblasts isolated from the lungs of patients with Idiopathic Pulmonary Fibrosis (IPF), a fibrotic lung disease of unclear etiology for which there is no definitive therapy. First, we examined survivin expression in lung tissue from patients with IPF and found that there is robust expression in the fibroblasts residing within fibroblastic foci (the “active” lesions in IPF which correlate with mortality). Next, we show that survivin expression is increased in fibroblasts isolated from IPF lung tissue compared to cells from normal lung tissue. Consistent with a role in fibrogenesis, we demonstrate that TGF-β1 increases survivin expression in normal lung fibroblasts. Finally, we show that inhibition of survivin enhances susceptibility of a subset of IPF fibroblasts to apoptosis. Collectively, these findings suggest that increased survivin expression represents one mechanism contributing an apoptosis-resistant phenotype in IPF fibroblasts.
Treatment of Idiopathic Pulmonary Fibrosis - An unmet clinical need
Philip M Short,Nik Hirani
Scottish Universities Medical Journal , 2012,
Abstract: Idiopathic Pulmonary Fibrosis (IPF) is a progressive lung condition with no clear underlying definitive cause. This article will discuss previous landmark studies in IPF and review current and potential future treatment options.
Towards a better diagnosis of idiopathic pulmonary fibrosis
D. Valeyre
European Respiratory Review , 2011,
Abstract: Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias, and poses significant clinical challenges. IPF diagnosis is based on clear-cut computed tomography (CT) and histopathological criteria, in an appropriate clinical context. The diagnostic criteria include: 1) exclusion of known causes of interstitial lung disease (including connective tissue disease); 2) usual interstitial pneumonia pattern on high-resolution CT in patients not subjected to surgical lung biopsy; and 3) specific combinations of high-resolution CT with pathological patterns in case of surgical lung biopsy. Improved diagnosis of IPF may help physicians to reduce the delay before an accurate diagnosis is made and increase patient awareness and access to adequate information, follow-up and treatment.
Anti-cytokine therapy in fibrosing alveolitis: where are we now?
Ann Millar
Respiratory Research , 2000, DOI: 10.1186/rr2
Abstract: Idiopathic pulmonary fibrosis (IPF) is a chronic debilitating illness that leads inexorably to respiratory failure and death in most patients [1]. The 5-year mortality approaches 50% in most studies [2]. The efficacy of any treatment is questionable and this can lead to a rather nihilistic approach. Received wisdom suggests that this is a rare disease occurring mainly in elderly men and for which currently available treatment is often ineffective and can induce side effects that are worse than the condition itself. The symptoms of gradually increasing breathlessness are non-specific and often attributed to `old age', leading to presentation at a point that can be late in the natural history of the condition. Current therapy is usually corticosteroids with or without some form of immunosuppressant. However, the data upon which this is based are limited. The fact that these treatments are still widely used reflects the difficulty of a palliative approach for clinicians and patients in such a distressing condition.The available studies of treatment for IPF are in small numbers of patients and are rarely controlled trials [3,4,5,6,7,8,9,10,11,12,13,14]. On reviewing these studies, it is apparent that some of the patients had collagen vascular disease and therefore did not have IPF. A significant number of the responders were less than 50 years old and were female, which is atypical. Histological data on these subjects were limited. The recent reclassification of interstitial lung diseases by histological features has shown a clear association between subtypes and response to treatment. Usual interstitial pneumonia (UIP) is the histological pattern that identifies IPF with little response to treatment [15,16]. Overall, these data suggest that those patients who responded to treatment in a previous trial might have had non-specific interstitial pneumonia (NSIP) rather than UIP. This exemplifies problems in the design and practice of appropriate trials in IPF owing to the
Calidad de vida en pacientes con fibrosis pulmonar idiopática
Tabaj,G; Quadrelli,S; Grodnitzky,L; Sinagra,P;
Revista americana de medicina respiratoria , 2012,
Abstract: idiopathic pulmonary fibrosis (ipf) is a chronic disease that primarily affects adults from the fifth decade of life. it is characterized by progressive dyspnea, sustained functional decline and survival of less than three years after diagnosis. so far, the treatment has focused on improving survival, but for many patients, an improvement in quality of life (qol) is a more realistic goal. despite the significant impact the disease has on physical, emotional and social features of the patient's life, research focused on qol is very limited. currently, there are no fully validated instruments appropriate for the specific evaluation of qol in patients with ipf. generic measurement tools used in patients with ipf include the who 100 items instrument (whoqol-100), the quality scale of well-being (qwb) and the 36 items instrument (sf-36), but none of them was designed specifically for patients with ipf. in the reviewed studies, patients with ipf showed impairment of the health related qol in almost all aspects of life, but those related to physical functioning, symptoms and levels of independence were the most affected ones. more research is needed to learn about how this disease affects ipf patients, whether they adapt themselves to their disease over time, and if so, how. furthermore, research needs to show whether the qol measurement tools available are reliable and valid measures for health related qol over time among patients with ipf.
Exacerba??o aguda da fibrose pulmonar idiopática
Melo,Natália; Damas,Carla; Moura,Concei??o Souto; Morais,António;
Revista Portuguesa de Pneumologia , 2009,
Abstract: some patients with idiopathic pulmonary fibrosis (ipf) have disease accelerated deterioration without identifiable cause referred as ?acute exacerbation? or ?accelerated stage?. it is characterized by severe worsening of dyspnea, hypoxemia and new or progressive opacities on imaging studies. the typical histological findings are diffuse alveolar damage in addition to the features of usual interstitial pneumonia pattern. mortality in this clinical entity is very high and no efficacious therapeutic have been described. the authors describe the clinical, functional and radiological features, treatment and evolution of five patients with ipf acute exacerbation. a discussion will be carry out concerning the ipf acute exacerbation usual features comparing with the alterations noticed in those patients.
The role of chemokines and cytokines in lung fibrosis
M. P. Keane
European Respiratory Review , 2008,
Abstract: Idiopathic pulmonary fibrosis (IPF) exhibits a complex and poorly understood pathogenesis. Overt inflammation in the lungs of patients with established IPF is absent, and classic anti-inflammatory therapies are inefficacious; however, inflammation may contribute to the disruption of the normal alveolar architecture, allowing interaction between fibroblasts and the epithelium. The polarisation of the inflammatory response toward a type-2 helper T-cell phenotype may also be important in the development of pulmonary fibrosis, as fibroproliferation could be favoured over repair. Furthermore, evidence has emerged regarding an imbalance between angiogenic and angiostatic chemokine levels, leading to an overall angiogenic pattern of expression in both animal models and tissue specimens from IPF patients. The precise role of vascular remodelling in IPF remains to be determined. Therefore, numerous questions exist regarding the role and importance of chemokines and cytokines in pulmonary fibrosis. Further investigation is required to facilitate the elucidation of IPF pathogenesis and identification of novel targets for treatment of this dismal disease.
Pneumonias intersticiais idiopáticas: Uma revis?o da literatura
Mota,Paulo José de Lima;
Revista Portuguesa de Pneumologia , 2006,
Abstract: interstitial idiopathic pneumonias are a group of diseases whose rarity and variety of clinical, radiological and pathological descriptions creates difficulties in study and management. an example of this is the classification method for this group, with only 2002 seeing some consensus. the aim of this article is to review the main literature to contribute to an understanding of this subject.
The Expression of Matrix Metalloproteinases and Tissue Inhibitors of Metalloproteinases in Idiopathic Interstitisal Pneumonia  [PDF]
Ji Young Shin, Yu Jin Kim, Sun Young Kyung, Seung Yeon Ha, Sung Hwan Jeong
Open Journal of Respiratory Diseases (OJRD) , 2014, DOI: 10.4236/ojrd.2014.43014
Background: Idiopathic interstitial pneumonia is characterized by fibroblast proliferation and extracellular matrix (ECM) accumulation. Matrix metalloproteases (MMPs) and tissue inhibitors of metalloproteases (TIMPs) have been shown to regulate remodeling of the ECM, which indicates that they are important factors in the process of lung fibrosis. Therefore, we evaluated the expression of MMPs and TIMPs in tissues obtained from patients with idiopathic interstitial pneumonia and control tissues. Methods: Thirty-seven patients who were diagnosed with IIP (22: IPF, 13: NSIP, 2: COP) and 5 controls were enrolled in this study. The MMP-2 and -9 activity in lung tissue obtained from these patients was analyzed using gelatin zymography and the levels of TIMP-1 and -2 were measured by western blotting. We also evaluated the expression of MMP-2 and -9, as well as that of TIMP-1 and -2 in lung tissue using immunohistochemistry. Results: The levels of MMP-2 and MMP-9 were significantly increased in patients with IPF compared to those with NSIP and COP. The activities of TIMP-1 and -2 were also higher in patients with IPF than NSIP/COP patients and control subjects. There were no significant differences observed in the activities of MMPs and TIMPs obtained from patients with NSIP/COP and control subjects. The immunohistochemical analysis showed that TIMP-2 and MMP-2 were strongly stained at the fibroblasts of the fibroblastic foci in patients with IPF. Conclusions: These results suggest that over-expression of gelatinases and TIMPs in patients with IPF are important factors in the irreversible fibrosis that is associated with lung parenchyma.
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