View clinical trials related to Pulmonary Fibrosis.
Filter by:To evaluate the efficacy and safety of 26-weeks of treatment with riociguat vs. placebo in patients with symptomatic PH (pulmonary hypertension) associated with IIP (idiopathic interstitial pneumonias).
Pirfenidone as anti-fibrosis drug developed in recent years demonstrated the potential anti- fibrotic effect, but so far there were no domestic studies about pirfenidone's efficacy and safety evaluation in china. The aim of this study was to evaluate the efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) through the observation of a large sample of clinical cases.
Scarring of the lungs is common in patients with scleroderma and is one of the main causes of death. Patients with scleroderma very frequently have problems with their gullet (esophagus), the food pipe that leads into the stomach. Normally, a small circular muscle at the base of the esophagus opens to allow food to pass into the stomach and closes to keep the digestive fluids from flowing back up into the gullet. In patients with scleroderma, the muscle may become weak and no longer close properly. Gastroesophageal reflux (GER) is the medical term for reflux of stomach contents into the esophagus. Our hypothesis is that small amounts of GER can move back up into the esophagus and get inhaled into the lungs, and may be one of the triggers for lung scarring. We propose to look for certain substances normally only found in the stomach in the "exhaled breath condensate" which is collected by breathing comfortably into a cooled cylinder, allowing the breath to condensate. In a smaller group of patients, we also plan to perform a bronchoalveolar lavage, a more widely studied test in which a small amount of fluid is introduced into a small part of the lungs through a fine tube, and then removed for examination, to evaluate whether the two tests provide similar measurements. We will also evaluate the correlation between these molecules and other tests, including lung function, and markers of lung scarring activity, and tests to look at how the esophagus is working so that we can get a clearer picture of how this affects patients' daily lives. Finally, we will be following up patients over time with lung function to see whether evidence of GER into the lungs is linked with a greater likelihood of worsening of lung scarring in the future.
Despite intense research efforts and clinical trials, there is still no effective treatment that can prolong the survival of patients with IPF. Conventional therapeutic approach includes combination of corticosteroids, anti-oxidants, immunodepressants and immune modulatory anti-fibrotic agents to be discontinued 20 days before screening. The only, so far, therapeutic approach that has been proven effective in terms of prolonging patient's survival is lung transplantation. Nonetheless, not all the patients with IPF are eligible for lung transplantation; there is a significant proportion of these patients that finally succumb while waiting in a lung transplantation list. Therefore, there is critical need for more effective and reliable therapeutic modalities5. Adult Stem Cells (ASCs) seem to represent one of these. Therefore, it is conceivable to assume that adult-stem cells can be easily and safely be applied as a novel therapeutic agent in chronic and fatal lung diseases, including chronic obstructive pulmonary disease (COPD) and IPF. Therefore, there is an urgent need to provide a safe, effective and affordable treatment option for IPF patients. New diagnostic, prognostic and therapeutic strategies need to be developed to reduce the burden of IPF. Given the present lack of appropriate treatment adjunctive in the therapy of IPF, adipose derived stromal vascular fraction provides new opportunities for development of the same. MSCs are having anti-fibrotic activity and hence may be excellent source to tackle pulmonary fibrosis and hence could be explored for their therapeutic potential for treating Idiopathic pulmonary fibrosis. MSC's also display membrane-bound and insoluble secreted molecules involved with cell attachment to neighbouring cells and to the extra cellular matrix.18 This cell surface configuration may enable mesenchymal stem cells to home from bloodstream to mesenchymal tissue.14 As limited clinical information is available about use of SVF and MSC in the IPF patients hence this Open Label, Prospective, Randomized multi center comparative study has been undertaken to explore the tolerability & effectiveness of SVF in one treatment arm and MSC in second treatment arm in IPF patients. Adipose derived stromal vascular fraction and Mesenchymal Stem Cells has been found in preclinical studies to be safe and effective
There is plenty of evidence to suggest that the lung is not uniform. The internal surface area is 30 times that of skin, and the different bronchioles/bronchi/alveoli differ greatly in blood perfusion, temperature, oxygen tension, and pH. Also, particularly in the context of respiratory disease, notable differences are present in the structure of epithelial cells, cilia, production of mucus, and inflammatory/immune responses. All of these factors are known to impact the physiology of bacteria, yet, there is very little understanding of how they impact a) the presence/absence of particular bacterial species throughout the respiratory tract, or b) the metabolic processes used by these bacteria within the human host environment. A greater understanding of the relationships between environmental (chemical) gradients in the lungs of diseased patients (particularly those with cystic fibrosis) and the microbial communities that are present may lead to novel hypotheses about manipulation of the respiratory environment for therapeutic benefit. To investigate this further, the investigators propose to use explanted lung specimens from cystic fibrosis patients to test the following hypothesis: Hypothesis: In patients with cystic fibrosis, bacterial community composition, metabolism and environmental chemistry will vary depending on their spatial location within the airways.
The goal of this research study is to better understand current treatment practices for pulmonary exacerbations (lung infections) and whether the Cystic Fibrosis National Patient Registry (CFFNPR)can be used for this type of study.
Idiopathic pulmonary fibrosis (IPF) is a disease of unknown cause in which areas of normal lung tissue are replaced by scars. As a result it becomes harder for the lungs to extract oxygen from the air. IPF is commonly progressive, and around 50% of patients diagnosed with the disease die after approximately 3 years. The most common, troublesome symptoms of IPF are breathlessness on exertion, and cough. No drug treatments have been unequivocally shown to improve the death rate, or to significantly impact upon symptoms, in IPF. In recent years it has been recognised that cough can be caused by small amounts of liquid coming up from the stomach and "going down the wrong way" into the lungs, a process commonly known as "reflux". As liquid in the stomach is usually acidic, patients' lungs may repeatedly be exposed to small amounts of acid. Reflux is unusually common in IPF and could potentially contribute to the debilitating cough found with the disease. However there are many potential causes for cough in IPF. Stomach acid can be efficiently "switched off" by drugs called "proton pump inhibitors", one of which is called omeprazole. If reflux of stomach acid does contribute to cough in IPF, omeprazole might be expected to reduce cough. The purpose of this study is therefore to test whether omeprazole does reduce cough in patients with IPF. Sixty patients with IPF will be randomly allocated to have 3 months of omeprazole or a placebo. Neither the patient nor the doctor will be aware which treatment has been given, ie this is a randomised "double-blind", placebo--controlled trial. Patients' cough frequency will be measured before and after treatment and the change in cough frequency compared in those receiving omeprazole and those receiving placebo. Change in cough frequency is the main thing we aim to compare, but a range of other measurements will be assessed such as the numbers of patients eligible to take part, agreeing to randomisation and providing outcome data, patients' lung function, symptom scores, the amount of reflux, and the amount of inflammation in the lungs.
This is a clinical study to characterise the lung function, airway morphometry, pharyngometry and inhalation profiles in patients with mild to severe Idiopathic Pulmonary Fibrosis (IPF) over a period of up to 6 months. Inhalation profiles will be recorded from patients with IPF as they inhale during tidal breathing, and following two sets of instructions (maximal effort and 'long, steady and deep' inhalation), across a range of airflow resistances that reflect those of typical inhalers used to deliver medication to the lungs. Mouth and throat dimensions will be measured using an acoustic reflectance Pharyngometer. Measurements of lung function will be made using conventional sprirometry, plethysmography and diffusion, whilst Low Dose High Resolution Computed Tomography (HRCT) will be used to scan the airways at two lung volumes; functional residual capacity (FRC) and total lung capacity (TLC). Data from HRCT will be used to reconstruct airway morphometry, and model inhaled particle deposition within the lung. Overall, the study allows a further understanding of the IPF patient population, using the data to assist in the development of new inhaled products for this disease. Following up the patients with additional HRCT scans at 3 and 6 months will enable the sensitivity of CT based criteria of disease progression to be compared with lung function criteria. No investigational product will be used in this study.
Decrease in blood cell counts due to deficient bone marrow function, called bone marrow failure, as well as some lung diseases, called idiopathic pulmonary fibrosis, can be caused by genetic defects in telomere biology genes, eventually causing telomere erosion. These disorders are collectively termed "telomeropathies". There is evidence that male hormones may improve blood cell counts in marrow failure, and these hormones are able to stimulate telomerase function in hematopoietic cells in vitro. We propose this study to the use of male hormone in patients with aplastic anemia and pulmonary fibrosis associated with defects in telomeres.
Despite the implementation of modern public health interventions, 1 in 5 adults in the United States are either current or former smokers and remain at risk for the development of chronic lung diseases. It is unknown how or why any one individual smoker can develop a wide range of lung diseases including chronic obstructive lung disease and/or pulmonary fibrosis. The purpose of this protocol is to collect clinical data, blood, urine, and bronchoalveolar samples from smokers and non-smokers in an attempt to establish phenotypic clinical profiles that correspond to divergent pathways in the expression of such proteins as the transforming growth factor-beta1 (TGF-beta <=1). The information generated from this study will provide insight into the pathogenesis of smoking-related lung injury and potentially allow for the development of early therapeutic interventions.