Clinical Research Directory

Efficacy and Safety Study of Treprostinil Palmitil Inhalation Powder (TPIP) in Participants With Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

The primary objective of this study is to evaluate the effect of 24-weeks of once daily treatment with TPIP versus placebo on exercise capacity in adults with PH-ILD.

Fibrotic Disease Activity in Cardiopulmonary Disorders Using 18F-Fibroblast Activation Protein Inhibitor (18F-FAPI-74) PET/CT Imaging

Background: Injury or diseases of the heart and lung can sometimes cause scar tissue (fibrosis) to build up in those organs. Current imaging scans can see this scar tissue once it has formed, but researchers want to find a way to detect the fibrosis in its earliest stages, while there might still be time to prevent serious damage. A new tracer (a radioactive substance injected during imaging scans) may be able to help. Objective: To test a new tracer (18F-FAPI-74) during imaging scans in people with heart or lung disease. Eligibility: People aged 18 years and older with lung or heart disease that may cause scarring in those organs. Design: Participants will have 6 clinic visits over 2 years. Participants will be screened: They will have blood tests and tests of their heart and lung function. Those with heart disease will have a magnetic resonance imaging (MRI) scan of the heart. The study tracer will be used with positron emission tomography (PET)/computed tomography (CT) scans. The study tracer will be injected into a vein in the arm. Participants will lie on a padded bed that slides through a donut-shaped machine. Participants will have scans with the study tracer 2 times, 8 to 12 months apart. They will also have standard CT scans and blood tests during these visits. They will also have blood tests at 3 and 6 months between these visits. Participants will have a follow-up visit after 18 to 24 months. The study scans, MRI and standard CT scans, and lung function tests may be repeated....

Extension Study of Inhaled Treprostinil in Subjects With Fibrotic Lung Disease

Study RIN-PF-302 is designed to evaluate the long-term safety and tolerability of inhaled treprostinil in subjects with idiopathic pulmonary fibrosis or progressive pulmonary fibrosis.

A Study Evaluating the Safety and Efficacy of Inhaled AP01 in Participants With Progressive Pulmonary Fibrosis

A randomized, double-blind, placebo-controlled clinical study to evaluate the safety and efficacy of 2 doses of inhaled pirfenidone (AP01) versus placebo on top of standard of care in participants with PPF over 52 weeks.

Mycophenolate Mofetil in Systemic Sclerosis With Subclinical Interstitial Lung Disease

The goal of this pilot study is to assess the feasibility of a larger study on the efficacy of mycophenolate mofetil in people diagnosed with systemic sclerosis with mild lung involvement. Participants will be recruited over 12 months at 3 academic centers and assigned randomly to receive either mycophenolate mofetil or placebo, a look-alike substance that contains no active drug, for 96 weeks.

Clinical Efficacy of Telitacicept in Sjögren's Disease With Interstitial Lung Disease

This study aims to evaluate the efficacy Telitacicept in patients with primary Sjögren's Disease with interstitial lung disease

Aerobic Exercise in Patients With Pulmonary Hypertension

This study will determine if a rehabilitation exercise program can help people with pulmonary hypertension (PH) increase their physical activity. Patients with PH have an increase in blood pressure in the pulmonary blood vessels (artery, vein or capillaries) that leads to shortness of breath, dizziness, fainting and other symptoms. Healthy volunteers and people with pulmonary hypertension between 21 and 75 years of age may be eligible for this study. All participants undergo the following tests and procedures: * Medical history and physical examination * 6-minute walk test: Subjects walk as fast as they can for 6 minutes on a walking track to determine their ability to participate in physical activity. * Questionnaires: Subjects complete nine questionnaires related to their fatigue, daily physical activity, mood, and so forth. * Maximum treadmill test: The exercise begins at an easy level and gradually increases until the subject says he or she can no longer continue or the investigator decides it is not safe to continue. Subjects are fitted with a mask, electrodes and light sensors to measure how well the heart is working and how well the muscles use oxygen. Patients with pulmonary hypertension undergo the following additional procedures: * Activity monitoring: Patients wear a monitor for 3 days that measures movement and heart rate. * Group assignment: Patients are randomly assigned to Group 1 (education plus aerobic exercise) or Group 2 (education followed by exercise). * Group 1 patients will attend classes three days a week at either Inova Fairfax Hospital Pulmonary Rehabilitation Center or The National Institutes of Health for 10 weeks. Two sessions a week will include a 1 hour education session as well as a 30-45 minute track or treadmill exercise session. The third session will only include exercise. During the education patients will learn about a healthy lifestyle with pulmonary hypertension. After the 10 weeks of education and exercise, subjects repeat the 6-minute walk test, maximum treadmill test and questionnaires. * Group 2 patients participate in 2; 1-hour educational session at either the Inova Pulmonary Rehabilitation Center or The National Institutes of Health for 10 weeks. After the classes, they repeat the 6-minute walk test, maximum treadmill test and questionnaires. The following 10 weeks will consist of 3 days a week of 30-45 minute track or treadmill walking at either Inova or NIH, after which they again repeat the questionnaires, treadmill and walk tests.

Evaluation of the Reproducibility of the Automated Measurement of the Extent of ILD on Chest CT

In interstitial lung disease (ILD), the extent of ILD on chest computed tomography (CT) is recognized as an important prognostic factor. Automated tools are now available to quantify ILD, but there is a lack of data on the reproducibility of this measurement and therefore its accuracy. Therefore, the purpose of this study is to evaluate the variability of automated ILD quantification on chest CT. Reproducibility will be assessed by repeating chest CT scans and using different tools to measure the extent of disease.

Phase 2 Safety and Efficacy Study of Tulisokibart (MK-7240/PRA023) in Subjects With Systemic Sclerosis Associated With Interstitial Lung Disease (SSc-ILD) (MK-7240-007)

The purpose of this study is to assess the safety and efficacy of tulisokibart in participants with SSc-ILD.

Immunomodulatory Treatment of Interstitial Lung Disease Associated With Surfactant Related Gene Variants

Scientific justification : Variants in surfactant-related genes (SRG) explain approximately 6% of familial pulmonary fibrosis (FPF). The pathophysiology is unknown and seems to involve endoplasmic reticulum stress in type 2 alveolar epithelial cells. Variable improvement in the prognosis of childhood and adult interstitial lung disease (ILD) associated with a variant of a SRG, initially reported to be lethal within months of diagnosis, has been observed since the consensual use of prednisone, azithromycin and hydroxychloroquine targeting endoplasmic reticulum stress, without demonstration of the efficacy of any of these treatments alone or in combination. The investigators hypothesize that a treatment combining prednisone, azithromycin and hydroxychloroquine is safe and could improve the prognosis of adult patients with ILD associated with SRG variant. Main objective and primary endpoint : Main objective: Evaluate the efficacy of triple immunomodulatory therapy (prednisone, azithromycin and hydroxychloroquine) for 12 months in patients with ILD associated with a variant of a surfactant-related gene. Primary endpoint: Difference in forced vital capacity decline between the 2 groups at one year. Secondary objectives and endpoints : Secondary objectives: 1. tolerance of the triple therapy, 2. correlation between the respiratory, radiological and clinical functional response, 3. quality of life of the patients, 4. overall survival, transplant-free survival, exacerbation free-survival, hospitalization-free survival Secondary endpoints: 1. Clinical and biological tolerance (occurrence of an adverse effect during treatment), ECG (at 3, 6, 9, 12 months after randomization) (only HCQ or AZI patients) and ophthalmological (at one year after randomization) 2. Thoracic CT scan and PFT at 6 months and one year after randomization 3. Quality of life questionnaire (EORTC QLQ-C30, v3.0) at 3 months, 6 months, 9 months and one year after randomization, 4. Collection of vital status, lung transplantation, hospitalization for pulmonary and non-pulmonary causes and episodes of exacerbation at each visit until the end of follow-up 12 months after randomization. Design of the study : Multicenter, randomized, controlled, two-arm, parallel, open-label superiority study comparing triple immunomodulatory therapy (prednisone, azithromycin, and hydroxychloroquine) to standard of care Category : Category 2 Population of study participants: Patients aged over 18 years with ILD and SRG variant Number of participants included : 30 Design of the study : Multicenter, randomized, controlled, two-arm, parallel, open-label superiority study comparing triple immunomodulatory therapy (prednisone, azithromycin, and hydroxychloroquine) to standard of care.

Study Evaluating 68GA-FAPI-46 PET/CT in Patients With Interstitial Lung Disease

The purpose of this research is to better understand the distribution of an investigational radioactive imaging agent called 68Ga-FAPI-46 in the lungs of people with interstitial lung disease (ILD) during a Positron Emission Tomography/Computed Tomography (PET/CT) scan.

Global Utilization And Registry Database for Improved preservAtion of doNor LUNGs

The objective of this registry is to collect and evaluate various clinical effectiveness parameters in patients with transplanted donor lung that were preserved and transported within the LUNGguard system, as well as retrospective standard of care patients

Pulmonary Fibrosis Foundation Community Registry

Pulmonary fibrosis (PF) results from a diverse group of health conditions and affects the lives of patients (including those who are post lung transplant), caregivers and family members. The Pulmonary Fibrosis Foundation Community Registry will offer an online portal where participants can self-enroll and directly contribute information about their experience with PF to be compiled into a longitudinal data set for use by researchers.

Goals of Care Conversations Study

The long term goal is to improve quality of care in Veterans with serious illnesses by aligning medical care with Veterans' goals and values. The objective of this study is to use a sequentially randomized trial to determine what implementation strategies are effective to increase early, outpatient goals of care conversations. The study will use interviews with and surveys of medical providers, patients, and caregivers, along with medical record data. This work is significant because it tests ways Veterans can express their goals and preferences for life sustaining treatments and have them honored.

A Study of BIO 300 and Thoracic Radiation Therapy in People With Non-Small Cell Lung Cancer and Interstitial Lung Disease

The purpose of this study to find out whether giving BIO 300 in combination with thoracic radiation therapy is effective in preventing pneumonitis in people with non-small cell lung cancer (NSCLC) and interstitial lung disease (ILD).

SUPPORT-T in Patients With Progressive Pulmonary Fibrosis and Their Caregivers

This pilot randomized controlled trial evaluates the feasibility, acceptability, and accessibility of SUPPORT-T, a digitally delivered palliative care intervention for patients with Progressive Pulmonary Fibrosis (PPF) and their caregivers. The intervention includes a 9-week digital application guided by a nurse interventionist and quarterly virtual support groups over one year. Participants will be recruited from rural South Carolina and urban New York City. The study compares SUPPORT-T to Enhanced Usual Care and aims to inform a future multi-site trial.

A Study of Mosliciguat in PH-ILD

This is a Phase 2, randomized, double-blind, placebo-controlled, multi-center clinical study to evaluate the safety and efficacy of inhaled mosliciguat in participants with pulmonary hypertension associated with interstitial lung disease (PH-ILD).

Titrated Ambulatory Oxygen in Fibrotic ILD and COPD With Isolated Exertional Hypoxemia

Fibrotic forms of interstitial lung disease (ILD) and chronic obstructive pulmonary disease (COPD) are chronic lung disease which often affect how well oxygen can get from the lungs into the blood. Low blood oxygen levels often leads to shortness of breath which can affect patients' activity levels and quality-of-life. Many people with fibrotic ILD and COPD only have low oxygen levels when they are walking or exercising. Oxygen that is only used for walking or exercise is called ambulatory oxygen therapy (AOT). Laboratory studies suggest that AOT improves shortness of breath and exercise ability. However, real-world studies of AOT have not shown similar results. AOT can be given to patients through different types of equipment, most commonly oxygen tanks or portable oxygen concentrators (POCs). While previous studies have suggested that AOT does not significantly improve patients' breathing or activity in the real-world, these studies most often gave all participants the same amount of oxygen with the same device. However, patients with ILD and COPD often have very different oxygen needs during exercise, and POCs and oxygen tanks are very different in how oxygen is administered. This trial will test the feasibility of a study to determine whether real-world activity, symptoms, and quality-of-life are different with the use of different oxygen equipment when oxygen therapy has been adjusted to meet each participants' oxygen needs. A total of 24 participants (12 with fibrotic ILD and 12 with COPD) who only have low oxygen levels with activity will be randomly assigned to 2-week periods using either no oxygen therapy or oxygen delivered by oxygen tanks or POC. This trial will provide preliminary data to support a larger clinical trial to further test how different AOT equipment titrated to meet individual patients' needs may affect real-world outcomes in people with ILD and COPD.

Self-efficacy and Well-being of Patients With Fibrotic Interstitial Lung Disease

Patients with fibrotic insterstitial lung disease (ILD) participating in inpatient rehabilitation or in outpatient pulmonary care will be invited to participate in this observational study. Patients will fill out questionnaire regarding quality of life and symptoms at baseline and follow-up (at the end of rehabilitation or after the second outpatient ILD consultation). Additionally, clinical data obtained in clinical routine or for the IIP registry will be used for this project. The aim is to assess changes in self-efficacy and quality of life over time and to establish a control cohort for a later feasibility study on a educational and self-management intervention (currently in planning).

A Study to Assess the Efficacy and Safety of Treprostinil Palmitil Inhalation Powder (TPIP) in Participants With Progressive Pulmonary Fibrosis (PPF)

The primary objective of this study is to evaluate the effect of 52 weeks of once daily treatment with TPIP compared with placebo on lung function in adults with PPF.

Genentech Validation Tool for Pulmonary Fibrosis

Radiology is an important component of the multidisciplinary team. The primary goal of this project will be to create a tool using findings on chest computed tomography (CT) for nearly 300 patients with the most common types of fibrosis. The tool will be tested using chest CT scans of an additional 100 patients with the 3 most common diagnoses of fibrosis. The second objective will be to further validate the tool by performing a reader study with 3 co-investigating radiologists and ask them to look at 100 CT scans of patients with fibrosis without tool and then with tool and see if accuracy of diagnosis improves compared to the working diagnosis when using the tool.

Assessing Functional Capacity in Directly and Remotely Monitored Home-based Settings: A Protocol for a Multinational Validation Study in Individuals With Chronic Respiratory Diseases

The goal of this validation study is to provide further evidence of the metrological properties of the 6-minute stepper test in individuals with chronic obstructive pulmonary disease (COPD) or interstitial lung disease (ILD). The main questions it aims to answer are: 1. Is the 6-minute stepper test valid when compared to the 6-minute walk test and a maximal cardiopulmonary cycling test? 2. Does the 6-minute stepper test give the same results when conducted through direct (i.e., therapist in person) and remote (i.e., videoconferencing) monitoring? 3. Is the 6-minute stepper test safe when conducted at the home of the individual with direct (i.e., therapist in person) or remote (i.e., videoconferencing) monitoring? Participants will: * Conduct the 6-minute stepper test (several trials on separate days) * Conduct the 6-minute walk test (1 trial on 1 day) * Conduct a maximal cardiopulmonary test on a cycle ergometer (1 trial on 1 day) * Participate in a semi-structured interview to provide their feedback with regards to the 6-minute stepper test

DeciPHer-ILD: A Real-world Patient Registry in Group 3 Pulmonary Hypertension Associated With Interstitial Lung Disease (PH-ILD)

This is a prospective, real world, multicenter, registry of patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) and interstitial lung disease (ILD).

Reducing Respiratory Symptoms of Pulmonary Irradiation in Interstitial Lung Disease

In this double-blind phase II randomized controlled trial, patients with lung cancer or ≤2 oligometastatic pulmonary lesions and a concomitant diagnosis of ILD who are planned for radical Radiation Therapy (RT) will be randomized using a 2 x 2 factorial design to oral N-acetylcysteine (NAC) versus placebo, and also to short course corticosteroids versus placebo.