Active clinical trials for "Idiopathic Pulmonary Fibrosis"

The first-in-man study are designed as below to assess safety, tolerability, and preliminary pharmacokinetics of ZL-2102. Double-blind randomized, placebo-controlled ascending single oral doses (Part 1, ZL-2102-SAD); Open-label, randomized, 2-sequence, 2-period, 2-treatment crossover (Part 2, ZL-2102-FED); Double-blind randomized, placebo-controlled, ascending repeated oral doses for 14 days (Part 3, ZL-2102-MAD). A total of 104 subjects will be enrolled.

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.

Over time, patients with fibrosing or interstitial lung disease (ILD) can develop high lung blood pressures (pulmonary hypertension), and this is associated with poorer prognosis and survival. It is thought that development of PH contributes to the deterioration and death of patients with ILD. Endothelin-1 (ET1) is a substance contributing to the development of both PH and ILD. Bosentan is a drug blocking the action of ET-1 by binding to its receptors. Bosentan clearly benefits patients with PH of unknown cause, or related to other diseases (such as heart conditions, or HIV) both alone and in combination with other treatments. In patients with fibrosing lung disease and PH, there have been no controlled treatment studies. Clearly it is important to evaluate the effectiveness of bosentan in these patients. This study aims to determine the ability of bosentan to reduce high blood pressure in the lungs (pulmonary hypertension) in patients with scarring (fibrosing) lung disease. It is a placebo-controlled double blinded study for 16 weeks (and it is proposed to follow patients in a 16 week open-label phase with bosentan therapy).

Idiopathic pulmonary fibrosis (IPF) is a diffuse lung disease, associated with the histological appearance of usual interstitial pneumonia (UIP), with an inexorably deteriorating clinical course. Prognosis is poor, reported median survival is less than 3 years. The prevalence is estimated as being 3 to 10 per 100.000 in different Western populations. To date, no pharmacological therapy has been proven to alter or reverse the pathogenic process of IPF. Most treatments trials have been observational case series of small patient populations and very few have been randomized, prospective and placebo-controlled. Two recent Cochrane reviews investigated the role of corticosteroids and other immunomodulatory agents and concluded that there is no evidence for their use in IPF. Most current therapies are targeted to suppress the inflammatory component of the disease, based on the theory that it would be chronic alveolar inflammation which leads to parenchymal remodeling and fibrosis. Recently, a hypothesis that has gained acceptance suggests that fibrosis may result directly from alveolar injury, promoting an abnormal fibrogenic repair mediated by fibroblasts and myofibroblasts. One of the cytotoxic agents most widely used and better tolerated in the management of IPF is azathioprine. Based upon limited data available and from a single small high quality randomized controlled trial (RCT), this drug appears to confer, given in conjunction with prednisone, a marginal long term survival advantage. Since this combination therapy is associated serious adverse effect, we planned to design a trial of low dose corticosteroid and azathioprine versus placebo in management of IPF, evaluating progression-free survival. Our study hypothesis is: Combined therapy with azathioprine and corticosteroids improves progression-free survival in patients with the diagnosis of IPF.

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.

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.