Study of Denufosol Inhalation Solution in Patients With Mild Cystic Fibrosis Lung Disease
Cystic FibrosisThe purpose of this trial is to evaluate the safety and effectiveness of a one dose strength of denufosol compared to placebo in patients with mild CF lung disease.
Open Label Extension Study in Patients With Idiopathic Pulmonary Fibrosis Who Completed Protocol...
Idiopathic Pulmonary FibrosisThis Open-label extension study in patients with Idiopathic Pulmonary Fibrosis who completed protocol AC-052-321 / BUILD 3 (NCT00391443) will asses the long term safety and tolerability of bosentan in patients with idiopathic pulmonary fibrosis (IPF).
Safety And Efficacy of BIBF 1120 in Idiopathic Pulmonary Fibrosis
Pulmonary FibrosisThe general purpose of this trial is to investigate the efficacy and safety of 4 dose strategies of BIBF 1120 treatment for 12 months, compared to placebo in patients with idiopathic pulmonary fibrosis. The primary objective of this study is to demonstrate whether at least one dose strategy is superior to placebo in patients with IPF, in modifying the rate of decline of Forced Vital Capacity (FVC). As a secondary objective, additional parameters will be assessed in order to differentiate between dose strategies on the basis of safety and efficacy
Safety and Efficacy Study of Depelestat in Acute Respiratory Distress Syndrome (ARDS) Patients
FibrosisLung Disease6 moreThe study is aimed to assess safety of Depelestat treatment, as well as efficacy on prevention and treatment of alveolar inflammation in early pulmonary fibrosis in patients suffering from persistent Acute Respiratory Distress Syndrome.
Three-Arm Study of the Safety and Efficacy of Pirfenidone in Patients With Idiopathic Pulmonary...
Idiopathic Pulmonary FibrosisThe objectives of this study are to assess the safety and efficacy of treatment with pirfenidone 2403 milligrams per day (mg/d) compared with placebo in patients with idiopathic pulmonary fibrosis (IPF), to assess the safety and efficacy of treatment with pirfenidone 1197 mg/d in patients with idiopathic pulmonary fibrosis and to characterize the pharmacokinetic disposition of pirfenidone in patients with idiopathic pulmonary fibrosis.
Study of GC1008 in Patients With Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic Pulmonary FibrosisThis study is designed to investigate whether GC1008, an antibody that neutralizes TGFb, is safe in treating patients with idiopathic pulmonary fibrosis. The highest dose without excessive side effects will be identified. Tests will determine how long GC1008 is in the body and how it is excreted.
A Study of the Safety and Clinical Effects of Interferon Gamma-1b in Patients With Idiopathic Pulmonary...
Idiopathic Pulmonary FibrosisStudy GIPF-002 is a phase 2 study designed to characterize the biologic and clinical effects of IFN-g 1b. The objective of the Study is to characterize the biologic and clinical effects of IFN-g 1b administered to patients with idiopathic pulmonary fibrosis (IPF). The Study will be conducted at multiple sites and enroll 30 patients with IPF who have failed treatment with corticosteroids.
Study Evaluating the Safety and Efficacy of Etanercept in Patients With Idiopathic Pulmonary Fibrosis...
Pulmonary FibrosisThe objective of the study is to evaluate the safety and efficacy of etanercept in comparison with placebo in a double-blind, parallel, randomized fashion in subjects with idiopathic pulmonary fibrosis (IPF) who failed previous therapy. The treatment period will be up to 1 year. The primary objective is evaluation of safety and efficacy. Secondary: The secondary objective is to evaluate quality of life (QoL) and pharmacokinetics (PK).
Study of INS37217 Inhalation Solution in Mild to Moderate Cystic Fibrosis Lung Disease
Cystic FibrosisThe purpose of this study is to assess the safety and effectiveness of multiple dosages of INS37217 compared to placebo over 28 days in subjects with mild to moderate cystic fibrosis (CF) lung disease. Study drug will be administered through a nebulizer (a device that delivers medication as a mist by breathing it in).
Oral Pirfenidone for the Pulmonary Fibrosis of Hermansky-Pudlak Syndrome
AlbinismInborn Errors of Metabolism3 moreHermansky-Pudlak Syndrome (HPS) is an inherited disease that results in decreased pigmentation (oculocutaneous albinism), bleeding problems due to a platelet abnormality (platelet storage pool defect), and storage of an abnormal fat-protein compound (lysosomal accumulation of ceroid lipofuscin). The disease can cause poor functioning of the lungs, intestine, kidneys, or heart. The most serious complication of the disease is pulmonary fibrosis and typically causes death in patients 40 - 50 years old. The disorder is common in Puerto Rico, where many of the clinical research studies on the disease have been conducted. Neither the full extent of the disease nor the basic cause of the disease is known. There is no known treatment for HPS. The drug pirfenidone blocks the biochemical process of inflammation and has been reported to slow or reverse pulmonary fibrosis in animal systems. In this study researchers will select up to 40 HPS patients diagnosed with pulmonary fibrosis. The patients will be randomly divided into 2 groups. The patients will not know if they are taking pirfenidone or a placebo "sugar pill". Group one will be patients who will receive pirfenidone. Group two will be patients who will receive a placebo "sugar pill" The major outcome measurement of the therapy will be a change in the lung function (forced vital capacity). The study will be stopped if one therapy proves to be more effective than the other.