View clinical trials related to Idiopathic Pulmonary Fibrosis.
Filter by: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.
- 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.
Idiopathic pulmonary fibrosis is a relentlessly progressive disease that is responsible for the deaths of over 5000 people in the UK each year. At present, despite a dramatic increase in clinical trials in the last decade, there are no licensed treatments for IPF. The pathogenesis of the condition remains incompletely understood, nonetheless there is good evidence to suggests that the condition arises as the consequence of an aberrant wound healing response in genetically susceptible individuals. Basic science research into IPF has identified a wide range of potential treatment targets. However, in many cases developing compounds to act against these targets, because of their role in normal wound healing, is limited by the possibility of major systemic side effects. The lung is highly amenable to topical therapy in the form of inhaled drug preparations and this route is utilised in the treatment of the majority of respiratory disease. The inhaled route offers a number of important potential advantages for administration of therapy to patients with IPF. Firstly, by limiting systemic exposure to drugs, the inhaled route offers the potential for achieving higher lung doses of drugs that might otherwise cause systemic toxicity. Secondly, inhaled treatment may more effectively reach the areas of abnormality in IPF, namely the hyperplastic epithelium and the underlying fibroblastic foci. Thirdly, the inhaled route offers an alternative to parenteral administration of compounds that are poorly absorbed through the gastro-intestinal tract e.g. monoclonal antibodies. It should be noted however, that the fibrosis in IPF develops peripherally involving the alveolar interstitium and the terminal bronchioles. Furthermore, the disease causes architectural destruction and distortion of the lung that is liable to alter the normal laminar flow of air (and inhaled particles) through the bronchial tree. It is therefore, by no means certain that it is possible to deliver inhaled therapies directly to regions of fibrosis in IPF. The feasibility of delivering inhaled drugs in IPF has not been previously studied. This research by assessing the effect of particle size on inhaled particle deposition and by relating to this the pharmacokinetic profile of salbutamol aims to validate the potential of the inhaled route in IPF. This study is an important precursor to the development of specific topical therapies for patients with IPF.
The purpose of this study is to determine the possible efficacy of low dose, orally administered interferon alpha in subjects with Idiopathic Pulmonary Fibrosis (IPF).
Primary objective of this study is to investigate the long-term tolerability and safety profile of BIBF 1120 on top of pirfenidone treatment in patients with Idiopathic Pulmonary Fibrosis who have completed a prior clinical trial of BIBF 1120 (1199.31). Secondary objectives are to assess effects on some efficacy criteria during long term treatment with BIBF 1120 on top of pirfenidone.
The primary objective of this study is to establish the feasibility and safety of infusions of placental Mesenchymal Stem Cells (MSC) from related or unrelated HLA identical or HLA mismatched donors in the treatment of Idiopathic Pulmonary Fibrosis (IPF). The secondary objectives are to document changes in lung function, 6 minute walk distance (6MWD), gas exchange and radiological appearance following infusion of MSC over a six month evaluation period.
- Pulmonary diseases are increasingly important causes of morbidity and mortality in the modern world. - Sildenafil, an orally administered a phosphodiesterase type 5 (PDE-5) inhibitor, targets the nitric oxide (NO) pathway. The drug was first approved for the treatment of Pulmonary Arterial Hypertension (PAH) in 2005. - The aim of the suggested study is to examine the acute effect of oral intake of sildenafil on exercise tolerance and functional capacity in Chronic Obstructive Pulmonary Disease (COPD), Idiopathic pulmonary fibrosis (IPF) and post Pneumonectomy patients. - The investigators hypothesize that oral ingestion of sildenafil prior the exercise may enhance exercise tolerance and improve function in COPD, IPF and post Pneumonectomy patients. - Patients and Methods: Sixty chronic lung disease patients males and females (aged 30 to 90 years) 20 COPD (GOLD III-IV) [9, 39] , 20 IPF and 20 post Pneumonectomy patients will be recruit to this study. - All subjects will carried out two maximal cardiopulmonary exercise tests (CPET) on bicycle ergometer in different days; 60 min after intake of placebo and 60 min after intake of 100 mg sildenafil (Pfizer, Sandwich, UK), in random order. - In first meeting prior exercise test at rest standard pulmonary function test, diffusion of CO, TLC and RV will be measured. In addition, Doppler Echocardiography and blood samples for NT-proBNP will be taken prior and post each CPET. - After 15-20 minute of passive recovery post exercise test all patients will perform 3 short functional tests including 6 minute walk test to assess functional capacity.
The primary objective of this study is to evaluate the safety and tolerability of subcutaneously (SC) administered multiple, escalating doses of BG00011 (a humanized monoclonal antibody directed against the alpha v beta 6 (αvβ6) integrin, formerly known as STX-100) in participants with IPF. The Secondary objectives are to estimate the pharmacokinetic (PK) parameters after the 1st dose and after the last dose of multiple, escalating doses of BG00011 in participants with IPF, to assess the immunogenicity of BG00011 in participants with IPF, and to assess the effect of BG00011 on biomarkers isolated from bronchoalveolar lavage (BAL) and peripheral blood in participants with IPF.