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Extracorporeal Photopheresis in Lung Transplant Rejection for Cystic Fibrosis (CF) Patients
Background/Rationale Acute rejection (AR) is common in the first year after lung transplantation. AR has usually been reversible with treatment, but it can trigger chronic rejection that is the leading causes of late morbidity and mortality. Extracorporeal photopheresis (ECP) has emerged as a promising treatment for chronic rejection. The investigators postulate that the immunoregulatory property of ECP could promote graft tolerance immediately after lung transplantation. Objectives The aim of this trial is to evaluate the safety and efficacy of ECP as induction therapy for prevention of AR in recipients affected with cystic fibrosis in...
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Families-At-risk for Interstitial Lung Disease Study
The interstitial lung diseases (ILDs) are a family of closely related lung conditions characterized by alveolar inflammation, injury, and fibrosis not due to infection or neoplasia. While previously considered to be rare, a recent nationwide study found that idiopathic pulmonary fibrosis (IPF), a fibrotic ILD with a median survival of only 3.8 years, affects nearly 0.5% of older adults in the U.S. While pirfenidone and nintedanib slow the progression of IPF, neither reverses fibrosis nor prevents progression of the disease,and no studies to date have tested interventions that prevent the development of fibrotic ILDs.
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Feasibility & Effect of a Tele-rehabilitation Program in Idiopathic Pulmonary Fibrosis (IPF)
Introduction Idiopathic Pulmonary Fibrosis (IPF) is a progressive fibrotic lung disease of unknown cause with a median survival of 3-5 years. As the disease progresses, worsening of lung function and gas exchange impairment cause hypoxemia during physical activity leading to a downward spiral; dyspnea and fatigue lead to a reduction in daily physical activities, exercise tolerance, muscle strength and quality of life. Problems reported by IPF patients are social isolation, increased level of dependency and immobility. There is no curative treatment for IPF, but pulmonary rehabilitation (PR) is recommended by expert opinion for the majority...
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Fibroblast Specific Inhibition of LOXL2 and TGFbeta1 Signaling in Patients With Pulmonary Fibrosis.
This is a two part study. In the first part, the pharmacokinetic profile of Epigallocatechin-3-gallate (EGCG) in normal human volunteers given a single oral dose will be determined to set the dose for the second part of the study. In the second part of this study, lung biopsy fragments and urine samples from patients with interstitial lung disease treated with EGCG will be evaluated in biochemical assays and compared to samples from untreated control patients.
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First in Human Study in Healthy Volunteers Followed With Dosing in Participants With Lung or Liver Fibrosis
First in Human single ascending dose followed by multiple ascending doses in healthy volunteers.
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Genentech MRI Study
The purpose of this study is being done to determine whether magnetic resonance imaging (MRI) using inhaled hyper-polarized 129 Xenon gas can help visualize impaired lung function to detect changes over time in Idiopathic Pulmonary Fibrosis (IPF) patients receiving approved IPF treatments. Subjects will undergo an approximately hour long comprehensive MRI protocol, including administration of multiple doses of hyper-polarized 129 Xenon. The subjects will have this initial study prior to initiation of IPF therapies. Then the subjects will have repeat studies at 3, 6 and 12 months following the initiation of therapy. Additional studies ...
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Genetic Polymorphisms in Idiopathic Pulmonary Fibrosis (IPF)
The purposes of this study are: - to determine if there are specific genetic traits that might explain why patients have developed pulmonary fibrosis; - to determine if specific genetic traits account for differing patterns of inflammation and scar tissue that has formed in the patient's lungs.
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Genetics of IPF - Screening Study for Family Members
This study seeks to screen first degree family members of people with Idiopathic Pulmonary Fibrosis (IPF) for the earliest signs of lung fibrosis.
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Genomic and Proteomic Analysis of Disease Progression in Idiopathic Pulmonary Fibrosis (IPF)
The purpose of the study is to identify genetic and biologic markers that may predict the loss of lung function due to idiopathic pulmonary fibrosis. The studies will compare genetic and biologic markers of samples to changes in symptoms. The ultimate goal is to predict if or when patients are likely to experience a rapid decline in lung function due to disease progression.
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GKT137831 in IPF Patients With Idiopathic Pulmonary Fibrosis
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...
