Active clinical trials for "Pulmonary Fibrosis"

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive form of lung disease characterized by fibrosis of the supporting framework (interstitium) of the lungs. By definition, the term is used only when the cause of the pulmonary fibrosis is unknown ("idiopathic"). Microscopically, lung tissue from patients shows a characteristic set of histologic/pathologic features known as usual interstitial pneumonia (UIP). UIP is therefore the pathologic counterpart of IPF.Idiopathic pulmonary fibrosis is characterized by radiographically evident interstitial infiltrates predominantly affecting the lung bases and by progressive dyspnea and worsening of pulmonary function. No therapy has been clearly shown to prolong survival. The current strict definition of idiopathic pulmonary fibrosis provides a new focus for basic and clinical research that will improve insight into the pathogenesis of this disorder and stimulate the development of novel therapies. Pirfenidone has proven antifibrotic and anti-inflammatory properties in various in vitro systems and animal models of pulmonary fibrosis, although its precise mechanism of action remains unclear. It attenuates fibroblast proliferation, production of fibrosis-associated proteins and cytokines, and the increased biosynthesis and accumulation of extracellular matrix in response to cytokines such as transforming growth factor-β. It is also shown to slow tumor cell proliferation by inhibiting fibroblast growth factor, epidermal growth factor and platelet-derived growth factor. Pirfenidone has not been widely approved for clinical use in China, in this study, safety and efficacy were evaluated to see if pirfenidone has a significant advantage over placebo in terms of improving lung function and life quality etc. (see primary and secondary criteria) or slows down the deterioration of lung function in Chinese subjects diagnosed with IPF.

At present, a specific community based rehabilitation programme for lung or heart-lung transplant recipients does not exist. 160 hospitals throughout the United Kingdom (UK) offer pulmonary rehabilitation programmes. The programmes operate under evidence-based guidelines as outlined by the Chartered Society of Physiotherapy. Increasing evidence shows that rehabilitation programmes help improve performance, exercise endurance, and quality of life; and reduce symptoms and demand on health-care resources. This study proposes to compare the outcomes of lung and heart-lung transplant patients attending local pulmonary rehabilitation against others receiving the Trust's current document-based programme. The study is a randomized controlled trial: Control Arm: Those patients randomized to the 'control' arm will receive the Trust's standard rehabilitation programme that consists of an information pack supplied upon discharge. They will then complete and undertake the following tests: Short-Form 36 (SF 36; version 2) Questionnaire, Chronic Respiratory Questionnaire (CRQ-SR), Incremental Shuttle Walk Test and Spirometry forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC). The patient will follow the information contained in the information pack and the above tests and questionnaires will be repeated 6 months post discharge. Experimental Arm: A patient who is randomized to the 'experimental' arm will be asked to complete the following tests upon discharge: Short-Form 36 (SF 36; version 2) Questionnaire, Chronic Respiratory Questionnaire (CRQ-SR), Incremental Shuttle Walk Test and Spirometry FEV1 and FVC. Three months post discharge, the patient will be enrolled into a local pulmonary rehabilitation programme. The programme is typically structured to last 6-12 weeks. The above tests and questionnaires will be repeated 6 months post discharge. To measure the effectiveness of either the information pack or the rehabilitation programme the following endpoints will be subjected to analysis in both the experimental and the control group: Short-Form 36 Questionnaire; Chronic Respiratory Questionnaire; Incremental Shuttle Walk Test; Borg Scale; Spirometry FEV1 and FVC; Hospital re-admission rates and mortality rates. The undertaking of a multidisciplinary-led programme of rehabilitation facilitates a better quality of life than a document-based rehabilitation programme in lung and or heart-lung transplant out-patients. The aim of the study is to construct an optimal programme of rehabilitation in lung or heart-lung patients.

This study is designed to evaluate the reproductibility and the performance of the 3 minutes sit-to-stand test in patients with idiopathic pulmonary fibrosis. To do this, the investigators are recruiting 40 patients with idiopathic pulmonary fibrosis in 2 centers (Grenoble university hospital and Lyon university hospital). Patients had to achieve an effort test on a cycle ergometer. 2 visits are planned in the hospital. During each visit, patients will perform a 3 minutes sit-to-stand test, a 1 minute sit-to-stand test and a 6 minutes walk test. During the second visit, patients will also perform a 3 minutes sit-to-stand test with measurement of oxygen uptake. The investigators will then analyse the results by comparing numbers of cycle, functional response and symptoms during the 3 minutes sit-to-stand test of the 2 visits. The investigators will also compare the functional response obtained during the 3 minutes sit-to-stand test, the 1 minute sit-to-stand test and the 6 minutes walk test. Finally, the investigators will compare the maximal values of oxygen uptake, respiration rate and expired volume obtained during the 3 minutes sit-to-stand test to the effort test on cycle ergometer.

Idiopathic pulmonary fibrosis (IPF) is a chronic disease, leading to poor lung function with a median survival of 2-3 years. Acute exacerbation of idiopathic IPF is a complication associated with a mortality rate > 50%. So far, the appearance of an acute exacerbation is unpredictable. Worsening of the IPF accompanies with a decrease of the FVC-value, the lung capacity. So far, studies are missing investigating the correlation between a decrease of the FVC-value and emerging acute exacerbations. Therefore, this study uses daily home spirometry to investigate that correlation. With this study the investigators hope to determine acute exacerbations early and treat patients early.

High resolution computed tomography (HRCT) plays a major role in the management of idiopathic pulmonary fibrosis (IPF) by identifying characteristic lesions of usual interstitial pneumonia (UIP). Though HRCT is the standard reference to describe pulmonary structural alterations using a non invasive technique, it is nonetheless a radiating exam which provides limited functional information regarding inflammation. In this trial, the investigators aimed to evaluate whether MRI (Magnetic Resonance Imaging) using ultra-short echotime could be an alternative to HRCT in the assessment of the four morphological criteria required to define an UIP pattern. The investigators also planned to study the clinical value of the additional informations derived from MRI such as contrasts and lung perfusion using functional MRI.

The purpose of this study is to determine whether magnetic resonance imaging (MRI) using inhaled hyperpolarized 129Xe gas, and conventional contrast can help visualize impaired lung function and detect changes over time in patients receiving treatment as well as those who don't. 129Xe is a special type of xenon gas and when inhaled during MRI may be able to show areas of abnormal thickening of parts of the lungs. These images combined with images taken with injected contrast agents or other types of MRI may provide a better way to look at lung structure and function in patients with IPF. The ultimate goal is to predict how a particular patient might respond to a particular therapy and to observe such responses earlier than conventional tests. The investigators anticipate that the images acquired in this study will provide more specific information about lung disease than standard lung function tests. The use of 129Xe MRI is investigational. "Investigational" means that these tests have not yet been approved by the US Food and Drug Administration and are being tested in research studies like this one. In addition, standard MRI with contrast is not typically done as standard of care for monitoring progression of IPF, therefore, its use in this study is also considered investigational. Healthy volunteers are being asked to participate in this study because the investigators need to develop a database of functional images that are representative of healthy lungs.

RATIONALE: Losartan potassium may be effective in treating pulmonary fibrosis caused by radiation therapy in patients with non-small cell lung cancer. PURPOSE: This clinical trial is studying losartan to see how well it works in treating pulmonary fibrosis caused by radiation therapy in patients with stage I, stage II, or stage III non-small cell lung cancer.

This study is investigating the reliability of the 4 metre gait speed test (4MGS) in patients with a lung disease called Idiopathic Pulmonary Fibrosis (IPF).

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with a high mortality. Health-related quality of life (HRQL) is impaired in patients with IPF. Little is known about the properties of recently developed HRQL questionnaires and about the longitudinal changes in HRQL, including factors with an impact on HRQL. Comorbidities have an impact on patients with IPF, but reports differ in incidence and prevalence. The impact of comorbidities on HRQL and disease progression has only been studied sparsely. Also, the association between biomarkers and disease progression need to be examined further.

Fibroproliferative diseases, including pulmonary, cardiac and vascular fibrosis share common pathogenetic mechanisms. Furthermore, cardiovascular comorbidities are frequently found in patients with IPF. However, the prevalence of cardiac and vascular fibrosis in patients with IPF have yet to be determined. Main Purpose of this study is to evaluate, with non-invasive methods (echocardiogram, endothelial function and pulse wave velocity) and blood biomarkers (galectins-3, osteopontin, periostin and pro-BNP), the presence of vascular fibrosis (vascular rigidity and endothelial function) and cardiac fibrosis (prevalence of HFpEF - Heart Failure with Preserved Ejection Fraction) in patients with idiopathic pulmonary fibrosis (IPF), compared to healthy controls.