View clinical trials related to Pulmonary Fibrosis.
Filter by:To study the safety and effectiveness of multiple-doses of tralokinumab on pulmonary function in adults with mild to moderate idiopathic pulmonary fibrosis (IPF). IPF is a chronic, progressive, irreversible, and usually fatal lung disease of unknown cause.
The purpose of this study is to assess whether 6% hypertonic saline (HS) is a safe and effective preventive therapy in newborns and infants with cystic fibrosis (CF).
The aim of this extension trial is to assess the long-term safety of BIBF 1120 treatment in patients with Idiopathic Pulmonary Fibrosis who have completed one year treatment and the follow up period in the double-blind phase III placebo controlled parent trials (1199.32 and 1199.34), who wish to continue treatment with BIBF 1120.
The purpose of this research study is to learn about the safety of transplanting lungs obtained from non-heart-beating donors (NHBDs) that have been ventilated (attached to a breathing machine or ventilator to deliver oxygen) and perfused with a lung perfusion solution (Steen solution™, made by Vitrolife). This ventilation and perfusion will be done outside the body (ex-vivo) in a modified cardiopulmonary bypass circuit (the kind of device used routinely during most heart surgeries). The purpose of performing ex-vivo perfusion and ventilation is to learn how well the lungs work, and whether they are likely safe to transplant.
Primary Objective: To assess in adult patients with Idiopathic Pulmonary Fibrosis (IPF) the safety and tolerability of ascending doses of SAR156597 administered subcutaneously (SC) once weekly over a 6-week period. Secondary Objectives: To assess in adult patients with IPF: - The pharmacodynamic effects of SAR156597, as measured on pulmonary function tests (PFTs), pulse oximetry and patient reported outcome and peripheral blood biomarkers. - The trough plasma concentrations of SAR156597 - The potential immunogenicity of SAR156597.
This is a randomized, multi-center, open-label Phase II clinical trial to determine the efficacy of combined plasma exchange (PEX), rituximab, and conventional corticosteroid administration, in comparison to corticosteroids alone, among patients with acute Idiopathic Pulmonary Fibrosis (IPF) exacerbations. The investigators central hypothesis is that antibody-mediated autoimmunity can play an important role in IPF exacerbations. The investigators propose to test our central hypothesis by establishing the efficacy of autoantibody removal by plasma exchange (PEX), in conjunction with B-cell depletion by rituximab to deplete immunoglobulins and minimize their further production, among patients with acute IPF exacerbations. The primary goal of this randomized, multi-center, open-label Phase II clinical trial is to determine effects of combined plasma exchange (PEX), rituximab, and conventional corticosteroid administration on selected, relevant immunological parameters, in comparison to effects of steroids alone, among AE-IPF patients. The investigators anticipate the findings of this will lead to larger incremental trial(s) to determine actual clinical efficacy of this treatment. A total of 40 subjects will be enrolled in this multi-center trial from 5 participating medical centers.
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.
This study is an observational and retrospective study of patients with pulmonary fibrosis associated or not with telomerase mutation. The purpose of this study is to describe in detail the cases with telomerase mutation in terms of features on CT scan, respiratory function and evolution, in comparison to control subjects with idiopathic pulmonary fibrosis and no telomerase mutation identified or family history.
- Idiopathic pulmonary fibrosis (IPF) is a chronic progressive, fibrosing parenchymal lung disease with unknown etiology. The prevalence of IPF is estimated between 2-29 cases per 100,000 in general population with median survival rates of 2-5 years from time of diagnosed. - Dyspnea and chronic dry cough are the prominent symptoms in those patients which cause to impaired functional capacity and quality of life (QOL). - Drug therapy usually is ineffective approach, what makes a call for an effective treatment. - Pulmonary Rehabilitation (PR) had been founded as safe and effective treatment in increasing functional capacity, decrease symptoms and improve QOL mainly among Chronic Obstructive Pulmonary Disease (COPD). However, recently growing evidence also supports the efficiency of PR in IPF patients. - The Aim of the suggested study is to examine the effect of exercise training (ET) at ambulatory pulmonary rehabilitation among IPF patients. - The investigators hypothesize that ET at PR program will increase functional capacity, will decrease level of dyspnea and improve QOL in IPF patients. - Patients and Methods: - 40 IPF patients males and females (aged 30-90 years old) will be recruiting to this study. After clinical assessment they will be randomly allocated to PR group n=20 or to Control group n=20. - All patients will undergo a clinical assessment including medical history, risk factors for IPF and physical examination. On the first meeting at baseline and within one week post intervention (PR) the following measurements will be made: dyspnea score, QOL and physical activity level questionnaires. In addition, anthropometric measurements, echocardiography, blood samples, pulmonary function tests, cardiopulmonary exercise test, battery of functional test and 6 minute walk test (6MWT). - The PR group will participate in 12 weeks of supervised group's ET program. The training program will be consisted two 6 week blocks of 60 min exercise bout twice a week. In the first block the patients will perform aerobic interval training with treadmill walking, cycling, and step climbing. In the second block, subjects will perform longer periods of continuous aerobic exercise, with resistance training by step climbing, unsupported arm/leg exercises with and without dumbbells (0.5-1 kg), and supporting body weight over a chair. Pulmonary function test and 6MWT will be also performed after completing first 6 week block. The control group will be assessed at baseline and after 12 weeks without participating in PR program.
Idiopathic pulmonary fibrosis (IPF) is an illness characterized by progressive decline in lung function and premature death from respiratory failure. Fibrocytes are a novel population of bone marrow-derived circulating progenitor cells that have been shown to traffic to the lungs and contribute to fibrosis in animal models of pulmonary fibrosis, and whose numbers correlate with the degree of fibrosis and with survival in human pulmonary fibrosis. The investigators propose to test the hypothesis that therapy with the mTOR inhibitor, sirolimus, reduces the number of circulating fibrocytes in patients with IPF. The investigators propose to test this hypothesis in short-term pilot trial of sirolimus in patients with IPF to determine its effect on the number and phenotype of circulating fibrocytes.