View clinical trials related to Pulmonary Fibrosis.
Filter by:This study is meant to compare the amount of oxygen required for hypoxemia relief between current standard of care (oxygen only) and oxygen with the addition of high flow air for Chronic Obstructive Pulmonary Disease (COPD), Interstitial Lung Disease (ILD), and Pulmonary Hypertension (PH) patients during rest. Subjects will be titrated from 0 L/min until they maintain 95% SpO2 for each of the following delivery methods: 1. Pulses of pure oxygen (control) 2. Constant high flow air with pulses of pure oxygen 3. Out of phase pulses of high flow air and pure oxygen
This is a multicenter prospective registry of IPF patients in South Korea. The Seoul National University Bundang Hospital is the coordination center for the Korean IPF Registry built by a collaboration of the Korean Interstitial Lung Diseases (ILD) Study Group.
This study aims to evaluate the differences between patient's and their physicians' perception of quality of life and the effect of disease severity and co-morbidities. Patients and physicians will complete two sets of questionnaires at an initial clinic visit and again six months later.
The objective of the current research project is, by using high quality Swedish registry data, to evaluate use, tolerance and effect of anti-fibrotic drugs in IPF-patients. Secondary study objectives are to determine the clinical profile, determinants of treatment adherence, long-term safety and to describe the patient journey from the first sign of disease to end of treatment.
Idiopathic pulmonary fibrosis (IPF) is a prototype of chronic, progressive, and fibrotic lung disease. It has been considered rare, with an incidence estimated to 11.5 cases per 100 000 individuals per year. Increasing rates of hospital admissions and deaths due to IPF suggest an increasing burden of disease. The median survival time from diagnosis is 2-4 years. Recently two disease-modifying therapies, pirfenidone and nintedanib, have been approved worldwide. Both drugs reduce the disease progression as measured by progressive decline in forced vital capacity (FVC), with a reduction of overall mortality showed by meta-analysis of phase III pirfenidone trials. However, progression of disease continues to occur despite the currently available drug therapy. Many patients die from progressive, chronic hypoxemic respiratory failure, or less frequently from acute exacerbation of pulmonary fibrosis. In these patients, no data are available to guide management between continuation of the prescribed antifibrotic drug, to switch to the other available antifibrotic drug, or to combine the available drugs. The combination of nintedanib and pirfenidone is not recommended outside clinical trials. However, although both antifibrotic drugs were developed and approved as monotherapy, two recent trials have suggested the feasibility and safety of combining them over a 12-24 weeks period. These results encourage further studies of combination treatment with pirfenidone and nintedanib in patients with IPF. Such study is timely, as there is a risk that clinicians facing the continued worsening of disease in patients receiving one of the available drugs may prescribe both drugs combined outside clinical trials, potentially exposing patients to a currently unknown risk. This study will evaluate the efficacy and tolerance of the combination pirfenidone and nintedanib as compared to a "switch monotherapy": i.e. switching from the current to the other of the two existing drugs prescribed as monotherapy, in patients who present chronic worsening IPF despite receiving either pirfenidone or nintedanib and as to a "control group": i.e.treatment still be on as before randomization (pirfenidone or nintedanib).
Evaluate the feasibility of performing a multi-elemental imaging analysis of lung specimens from patients with ILDs, with an technology named LIBS (Laser Induced-Breakdown Spectroscopy)
A placebo-controlled, multicenter, randomized trial to test GKT137831 in ambulatory patients with idiopathic pulmonary fibrosis. This drug is an inhibitor of nicotinamide adenine dinucleotide phosphate (NADPH) oxidase (NOX) isoforms. The investigators hypothesize the drug will decrease pulmonary injury due to reactive oxygen species (ROS) generated by NOX enzymes, which are believed to play an important role in the development of IPF. Treatment with GKT137831 could result in significant benefit for a lung disease that has, until now, been almost invariably inexorable. This clinical trial represents the bedside application of a series of NOX translational and basic studies and discoveries, over several years, from the laboratory of Dr. Victor Thannickal.
Molecular diagnosis of idiopathic interstitial pneumonias is an innovative way to potentially improve the diagnostic accuracy of surgical lung biopsies (SLBs), introducing molecular classifiers of idiopathic pulmonary fibrosis (IPF) vs. non-specific interstitial pneumonia (NSIP) vs. chronic hypersensitivity pneumonitis (CHP). The investigators hypothesize that pre-defined gene expression profiles previously identified on large lung explants can still be identified and reproducible on smaller, clinically available surgical lung biopsies (SLBs), and can be used to increase diagnostic accuracy during multi-disciplinary discussion. The investigators also hypothesize that the expression level of individual, preselected genes that accurately differentiate IPF from NSIP and CHP on lung explants can be reproduced on SLBs. The investigators will isolate RNA from SLBs obtained from patients with IIP and perform microarray analysis to verify the reproducibility of gene expression profiles on SLBs. Individual genes expression levels will be determined by RT-PCR. The diagnosis will be determined by MDD and further validated by prospective follow-up of patients for a period of 3 years. The investigators will assess the impact of molecular diagnostic techniques on interobserver agreement during multi-disciplinary discussion. The investigators will prospectively follow the clinical course of patients after SLB for a period of 3 years to validate the diagnosis, and asses the diagnostic accuracy of molecular techniques.
Idiopathic pulmonary fibrosis (IPF) is a lung disease that limits the ability to breathe enough for a good workout. One way to improve the exercise training is to reduce the number of muscles being trained together. By training one leg at a time, the patient does not have to breathe as much allowing each leg a better workout. Our groundwork suggests it may work in patients with IPF. This study will help decide whether one-legged exercise training is better at improving a patient's exercise endurance compared to the usual way of exercising with both legs at the same time.
The fibrotic interstitial lung diseases (fILD) are characterised by lung scarring, distressing breathlessness and poor health-related quality of life. Exertional desaturation (low blood oxygen during exercise) is a hallmark of fILD, occurring in over 50% of patients. It is sometimes treated with ambulatory oxygen therapy (AOT), which involves breathing supplemental oxygen during physical activity. However the absence of clinical trials has given rise to marked variations in policy and practice globally. Even where AOT is available, treatment adherence using the traditional delivery method of cylinder gas is poor. Recently new devices called portable oxygen concentrators (POCs), have become available, which are lighter and more maneuverable than a cylinder. This may enhance adherence and maximize treatment benefits. This trial will determine the clinical benefits and societal costs of AOT for people with fILD and exertional desaturation. A randomised controlled trial with blinding of participants, assessors and clinicians, and an embedded economic evaluation will be conducted. A total of 260 participants with fILD and exertional desaturation will be randomly assigned to use either AOT or air delivered using a POC for 6 months. If this trial demonstrates clinical and economic benefits of AOT then the findings can be rapidly translated into practice.