Clinical Research Directory

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....

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).

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

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.

Emapalumab MDA5 Rapidly Progressive Interstitial Lung Disease (RP-ILD) Study

This is a proof of concept study to determine if Emapalumab appears effective for the treatment of anti-MDA5 antibody positive rapidly progressive interstitial lung disease (MDA5 RP-ILD). Emapalumab is a medication that is currently used for a severe problem with the immune system, called macrophage activation syndrome, and this disease shares some similar features with MDA5 RP-ILD.

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.

Virtual Nurse and Social Worker Palliative Care Team to Improve Quality of Life in Veterans With Heart Failure or COPD

Many Veterans living with heart failure or chronic obstructive pulmonary disease (COPD) experience significant symptoms - such as breathlessness, fatigue, depression, and anxiety -that reduce their quality of life. Despite how common these symptoms are, they are often not adequately addressed in routine care. This study tests whether a virtual team of a nurse and social worker can improve quality of life, depression, anxiety, and other patient-reported outcomes for Veterans with heart failure or COPD who are at high risk of hospitalization or death. The nurse helps Veterans manage their most bothersome symptoms using a structured approach, and the social worker provides brief counseling to address emotional and psychological concerns. Both work closely with the Veteran's primary care provider and palliative care clinician to coordinate additional care as needed. The study also examines whether enhanced implementation support helps VA sites adopt the program more successfully compared to standard implementation support.

Treatment Response in Immune-mediated Myositis Associated Rapidly-progressing Interstitial Lung Disease

Idiopathic inflammatory myopathies (IIM) are a group of autoimmune conditions characterized by inflammation of muscles with possible extra-muscular manifestations which can include skin and interstitial lung disease (ILD). IIM-associated ILD carries poor prognosis. Particular subtypes of IIM such as anti-melanoma differentiation-associated protein 5 positive (anti-MDA5+) dermatomyositis with ILD are most commonly associated with rapidly progressive-interstitial lung disease (RP-ILD). RP-ILD is defined as worsening dyspnoea on exertion, hypoxaemia, and presence of newly emerging or expanding ground glass opacities on radiographic or computed topography of chest imaging excluding drug or infectious cause. Particularly, patients with anti-MDA5+ dermatomyositis often have RP-ILD with high mortality of over 60% in the first six months of diagnosis. The mainstay of treatment is immunosuppression though there has been no highly efficacious therapy proven to date. Therefore, the overall goal is to improve patient outcomes in IIM-associated RP-ILD including those with anti-MDA5+ dermatomyositis through the development of better treatment regimens. The objective of this research study is to evaluate the efficacy and safety of a combined immunosuppressive regime in patients with IIM-associated RP-ILD. The investigators hypothesize that the simultaneous inhibition of particular targets in the innate and adaptive immune system will improve efficacy and patient survival. The approach involves a combination of four immunosuppressive medications targeting different pathways implicated in IIM associated ILD. If successful, this study could contribute significantly to improving clinical outcomes for patients with IIM-associated RP-ILD.

FAPI PET for Lung Fibrosis

This is a prospective exploratory biodistribution study in patients with interstitial lung disease (ILD). The purpose of this research study is to determine where and to which degree the FAPI tracer (68Ga-FAPI-46) accumulates in normal and fibrotic lung tissues of patients with interstitial lung disease. The study will include patients with interstitial lung disease who have or will initiate a new ILD medication OR will undergo tissue biopsy or surgery of the lung. The study will include 30 patients, the upper limit for PET imaging studies conducted under the Radioactive Drug Research Committee (RDRC) purview. Participants will be injected with up to 7 mCi of 68-GaFAPi and will undergo one PET/CT scan and one High Resolution CT of the lungs. The study is sponsored by Ahmanson Translational Theranostic Division at UCLA.

PRospective phenotypIng and Multi-omic Endotyping of Progressive Pulmonary Fibrosis

This is a prospective, observational cohort study. Participants with non-idiopatic pulmonary fibrosis, interstitial lung disease (ILD) will be followed for 24 months to systematically collect clinical, imaging, and biospecimen data. The primary objective is to optimize progressive pulmonary fibrosis (PPF) classification and establish PPF incidence for key ILD subtypes. Additional exploratory objectives are to 1) Prospectively validate a novel PPF classifier and assess performance durability over time, and 2) Determine whether multi-dimensional PPF prediction outperforms component approaches.

Autologous Stem Cell Transplantation in Patients With Systemic Sclerosis

The purpose of this study is to determine whether a regimen of high-dose immunoablative therapy will demonstrate safety that is consistent or improved with other published regimens in SSc patients, while maintaining a treatment effect.

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.

Bio-Banking of Specimens for Advanced Lung Disease and Lung Transplant Research

A major goal of this protocol is to support biomarker studies in advanced lung diseases, lung transplantation care, and to improve our understanding of the effects of viral and other infectious exposures to outcomes in our lung transplant and ALD patient populations.

An Open-Label Extension 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 safety and tolerability of the long-term use of TPIP in participants with PH-ILD from Study INS1009-311 (NCT07179380).

Effects of Upper Extremity Aerobic Exercise Training in Patients With Interstitial Lung Disease

Severe dyspnea, cough, fatigue, restrictive type ventilation disorder, decreased pulmonary function, impaired gas exchange, decreased cardiovascular function and exercise intolerance are frequently encountered findings in patients with interstitial lung disease. It was demonstrated exercise training has beneficial effects in patients with interstitial lung disease. However, no study investigated the effects of upper extremity aerobic exercise training on outcomes in patients with interstitial lung disease.

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.

H01 in Adults With Interstitial Lung Disease (The SOLIS Study)

Background: Interstitial lung disease affects the tissues that aid the transfer of oxygen and carbon dioxide between the air and the bloodstream. The disease can cause fibrosis, a thickening and scarring of lung tissue. Fibrosis often continues getting worse, and most people with this disease die in 3 to 5 years. Objective: To test a study drug (hymecromone) in people with interstitial lung disease or lung fibrosis. Eligibility: People aged 18 years and older with interstitial lung disease or lung fibrosis. Design: Participants will have at least 7 clinic visits over 5 months. Participants will have screening and baseline visits. They will have blood tests and tests of their heart function. They will give a sputum sample. Other tests will include: Spirometry: Participants will breathe in and out through a mouthpiece to measure how much air they can hold in their lungs and how hard they can breathe. Diffusion capacity of lungs for carbon monoxide: Participants will breathe in a gas that contains a small amount of carbon monoxide. Then they will breathe through a mouthpiece. This test measures how well oxygen moves from the air into the blood. Resting energy expenditure. Participants will lie still for 30 minutes with a clear dome over their head. This test measures the calories their body burns at rest. 6-minute walk test. Participants will walk at their normal pace for 6 minutes. Their vital signs and blood oxygen levels will be checked. Hymecromone is a tablet taken by mouth. Participants will take 2 tablets every morning and 2 tablets every night for 12 weeks. Participants who decide to continue longer may enter an optional 12 week extension phase, for a maximum treatment time of 24 weeks. Tests will be repeated at study visits.

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.

Abatacept for the Treatment of Common Variable Immunodeficiency With Interstitial Lung Disease

There is no standard of care therapy for patients with granulomatous-lymphocytic interstitial lung disease (GLILD) seen in common variable immunodeficiency (CVID). Abatacept has recently looked promising for the treatment of patients with complex CVID. This study is a multi-site, phase II, randomized, blinded/placebo-controlled clinical trial in pediatric and adult subjects to determine the efficacy of abatacept compared to placebo for treatment of subjects with GLILD in the context of CVID. Funding Source - FDA OOPD

Validation of AI-Based Detection of Idiopathic Pulmonary Fibrosis in Serial Chest Radiographs: A Retrospective Longitudinal Study

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease of unknown cause with a median survival of only 3-5 years after diagnosis. Early detection and timely initiation of antifibrotic therapy may improve outcomes, but diagnosis is frequently delayed. Chest radiography (CXR) is widely accessible and cost-effective but has limited sensitivity for early interstitial opacity (IO), so radiologists may miss or delay documentation of relevant findings. This retrospective, single-center, observational cohort study evaluates whether an artificial-intelligence algorithm (VUNO Med-Chest X-ray) can detect interstitial opacity earlier than radiologists in the historical chest radiograph series of patients who were diagnosed with IPF. The cohort was identified via a April 2025 registry screening of patients carrying an IPF diagnosis at Chung-Ang University Hospital. For each patient, the date of the first AI-detected IO (using a pre-specified score cutoff) is compared with the date of the first radiologist-reported mention of interstitial/reticular opacity, across all chest radiographs obtained before the IPF diagnosis date, within a 15-year retrospective imaging window anchored to the April 2025 screening date (January 2010-April 2025). The study also explores patient characteristics that modify this lead-time difference and whether longitudinal AI IO-score trajectories are associated with mortality.

Advanced Imaging to Assess the Effect of Immunosuppression on Progressive Fibrosis

The purpose of this study is to investigate how immunosuppression treatment affects measurements of active collagen deposition using \[68Ga\]CBP8 positron emission tomography (PET) and tissue injury using dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in individuals with non-idiopathic pulmonary fibrosis interstitial lung disease (non-IPF ILD).

Study of the Efficacy and Safety of Inhaled Treprostinil in Subjects With Progressive Pulmonary Fibrosis (TETON-PPF)

Study RIN-PF-305 is designed to evaluate the safety and efficacy of inhaled treprostinil in subjects with progressive pulmonary fibrosis (PPF) over a 52-week period.

Interstitial Lung Disease Research Unit Biobank

Establish a interstitial lung disease (ILD) registry and biorepository to lead towards a further understanding of the disease.

Evaluation of the Effectiveness, Safety, and Cost of a Smart Home-based Hospital System for Patients With Interstitial Lung Disease: Prospective Multicenter Randomized Controlled Trial

This study aims to analyze the effectiveness/safety/cost of the Smart Homespital system that provides a service that allows patients with interstitial lung disease to easily access and use biosignals generated using biosignal collection medical equipment at home.