Interstitial Lung Disease

This NIH-sponsored study aims to characterize three biomarkers derived from 129Xe gas exchange MRI and understand how they change in response to interventions. The study focuses on markers derived from the interaction of 129Xe with pulmonary capillary red blood cells RBCs, specifically RBC transfer MRI, cardiogenic oscillations in 129Xe-RBC signal amplitude, and the 129Xe-RBC chemical shift. The study population includes healthy volunteers, patients scheduled to undergo transfusion or phlebotomy, patients with dyspnea, interstitial lung disease ILD, idiopathic pulmonary fibrosis IPF, non-specific interstitial pneumonias NSIP, chronic hypersensitivity pneumonitis cHP, sarcoid, chronic thromboembolic pulmonary hypertension CTEPH, and acute pulmonary embolism.

Researchers are evaluating the safety, tolerability, pharmacokinetics PK, and immune response of a drug called SV001 compared to a placebo in healthy adult volunteers from China. This early phase 1, randomized, double-blind, and placebo-controlled study aims to understand how SV001 behaves in the body and whether it causes any adverse effects. The study focuses on adults aged 18 to 45 years who are in good health. Participants will receive a single dose of either SV001 or a placebo. The study uses a dose-escalation design, meaning doses may increase to assess safety. The trial is conducted at a single center and uses a quadruple-blind method where participants, care providers, investigators, and outcome assessors are unaware of which treatment is given. The intervention period includes administration of the study drug followed by monitoring. During the study, participants will undergo various assessments including monitoring for any treatment-emergent adverse events, measuring drug levels in the blood over time, and checking for immune responses to SV001. These evaluations will take place over approximately one year. Researchers will also monitor participants overall health, respiratory function, and conduct laboratory tests to ensure safety throughout the trial.

The GENESIS clinical study aims to map HLA genetic variation in the Greek population and evaluate possible correlations with selected underlying diseases. It is a multicenter, prospective, non-interventional clinical study targeting 12,000 subjects over an anticipated duration of 36 months, with the goal of creating a pilot HLA map for medical research and possible clinical applications. Each subject will complete one visit at a participating site and provide demographic information, including date of birth, gender, race, ancestry, height, and weight, as well as information about smoking or vaping, alcohol consumption, arterial blood pressure, diagnosed diseases, and current treatments. Recent clinical laboratory results from up to 12 months before sample collection may also be collected when available, including blood count, metabolic, liver enzyme, and biochemical parameters. Two buccal swabs will be collected from each subject for DNA extraction and HLA genotyping analysis. Selected DNA samples will also undergo low-pass whole genome sequencing to further investigate associations between the HLA region and autoimmune diseases. After the analysis is completed, an individualized ancestry report will be securely available to study subjects if they elect to access it.

Researchers are studying people with idiopathic pulmonary fibrosis IPF or progressive pulmonary fibrosis PPF who have previously taken nerandomilast in another study. The aim is to assess how well patients tolerate long-term treatment with nerandomilast and to evaluate whether it improves lung function and delays worsening symptoms, hospital visits, or death. This open-label extension trial is sponsored by Boehringer Ingelheim and focuses on treatment over an extended period. Participants take nerandomilast tablets for up to 1 year and 10 months while continuing their usual pulmonary fibrosis treatments. The study involves a single treatment group receiving the drug, and no placebo or comparison groups. Regular visits with doctors help monitor health and collect data during this extended treatment phase. Throughout the study, participants undergo regular lung function tests and health assessments to track any adverse events and changes in lung capacity. The main outcome measured is the occurrence of any adverse events for up to about 99 weeks. Secondary outcomes include changes in forced vital capacity and time to worsening of disease symptoms or hospitalization. The study includes ongoing safety monitoring with a total participation time of up to nearly two years.

This research aims to understand the risk factors, prevalence, and mortality associated with progressive pulmonary fibrosis PPF in patients who have interstitial lung disease ILD with pulmonary fibrosis visible on scans, excluding those with idiopathic pulmonary fibrosis IPF. It focuses on identifying connections between patient characteristics and progression of the disease over time. Participants will have initial assessments including collection of personal and medical histories, blood samples, detailed lung imaging high resolution computed tomography, lung function tests, and a 6-minute walk test. These evaluations will be repeated at 6 and 12 months, with additional blood samples and tests to monitor disease progression. Throughout the study, researchers will track lung function, exercise capacity, blood markers, and imaging results to measure disease progression and outcomes. The main goal is to link factors like age, gender, body mass index, and lung physiology scores to PPF progression over one year. The study extends up to 36 months to observe disease prevalence and long-term outcomes.

This research aims to evaluate deep learning algorithms designed to detect suspicious cell and tissue patterns for cancer in biopsies taken from peripheral lung nodules, masses, and mediastinal or hilar lymph nodes. The study uses the NIO Laser Imaging System during bronchoscopy procedures to image samples without traditional processing. It is a prospective, observational study focusing on rapid lung cancer detection with artificial intelligence assistance. The study includes four groups based on biopsy location and method transbronchial forceps biopsy of peripheral lung nodulesmasses, transbronchial needle aspiration biopsy of peripheral lung nodulesmasses, transbronchial needle aspiration biopsy of mediastinalhilar lymph nodes, and transbronchial cryo biopsy of peripheral lung nodulesmasses. Each biopsy procedure is performed as part of routine clinical care, and imaging is done in the procedure room using the NIO Laser Imaging System. Participants are adults scheduled for clinically indicated lung biopsies with an intermediate to high likelihood of lung cancer. The study tracks outcomes from enrollment until the end of each biopsy procedure. Researchers will assess how well the deep learning algorithms identify suspicious morphology during the procedure. Participants will be monitored during their procedures, and data will be collected to validate the technologys performance in detecting cancerous changes.

Pulmonary Alveolar Proteinosis PAP is a rare condition involving surfactant buildup in the lungs that causes breathing difficulties. This research aims to create a National PAP Registry to improve diagnosis and increase awareness of PAP. The study also focuses on defining the natural course of autoimmune PAP aPAP, developing a disease severity score, and testing new tools to assess lung disease severity and patient function. These efforts support advancing research and potential therapies for PAP. The study is divided into two parts. Part A establishes the National PAP Registry by enrolling participants with PAP to collect data through questionnaires and at-home blood collection using dried blood spot cards DBSC. This data helps validate new blood tests for diagnosing autoimmune PAP and identifying genetic risk factors. Part B reviews past medical records of aPAP patients, gathers patient input on symptoms and quality of life, develops a disease severity score combining patient reports and lung function data, and tests a mobile phone app to remotely measure exercise capacity. Participants contribute by completing questionnaires, providing blood samples by mail, and sharing medical records. Researchers evaluate blood tests for autoimmune PAP diagnosis, study disease prevalence, and analyze genetic risks. They also develop tools measuring how patients feel and function, including remote exercise testing. The study monitors participants over approximately five years, aiming to improve understanding, diagnosis, and patient-centered outcome measures for PAP.

Cystic fibrosis CF is a rare inherited disease that affects multiple organs, especially the lungs and digestive system. It is most commonly observed in Caucasians, but few cases have been reported among the large Chinese population. This research aims to accurately assess the prevalence of CF in China, along with the disease status, diagnosis and treatment approaches, quality of care, and health-related outcomes for Chinese patients. This study is observational and does not involve experimental treatments or interventions. It collects clinical information about Chinese patients diagnosed with CF, following the World Health Organizations diagnostic criteria. The study will monitor these patients over time to understand their lung function and related health measures. Participants will be evaluated for lung health changes, including spirometry tests measuring FEV1 and FVC, over a 10-year period. Data on genetics, clinical symptoms, treatments, and outcomes will be gathered to provide a comprehensive view of CF in China. Informed consent is required, and patients must be stable without recent respiratory infections to participate.

Pulmonary lymphangioleiomyomatosis LAM is a rare lung disease characterized by widespread cystic changes and almost exclusively affects women. This research aims to accurately determine how common LAM is in China and to evaluate the current disease status, diagnostic methods, treatment approaches, quality of care, and health outcomes for patients. The study is observational and will collect data over a period of four years from approximately 800 participants. This study is a national registry that gathers clinical information on Chinese women diagnosed with LAM. It will not involve any experimental treatments but will monitor patients health and treatments they receive in routine care. The primary outcome is to track the annual change in lung function, specifically measuring forced expiratory volume in one second FEV1 and forced vital capacity FVC. Secondary outcomes include changes in lung gas exchange ability, occurrence of serious health events such as bleeding or lung complications, pregnancy outcomes, tumors, lung transplants, deaths, and side effects from long-term rapamycin treatment. Participants will be followed for up to ten years with regular pulmonary function tests and health assessments. The study includes collecting data on major health events and adverse effects related to treatment over this time. Researchers will analyze these data to better understand LAM progression and outcomes in Chinese patients. The study requires participants to provide informed consent and be available for follow-up visits to contribute valuable information about this rare disease.

Acute respiratory failure is a serious medical emergency that can quickly lead to death without prompt treatment. Researchers are evaluating a new portable device designed to provide Continuous Positive Airway Pressure CPAP support outside of hospital settings. This device aims to be easy to use by non-medical individuals and to improve outcomes for patients experiencing respiratory failure. The study will test the devices effectiveness and usability in two groups healthy elderly adults who will self-apply the device, and hospitalized patients with mild oxygenation impairment. The device is designed to deliver positive end-expiratory pressure PEEP between 4 and 7.5 cmH2O, and its performance will be assessed in real-world clinical settings across three hospitals. Participants will be monitored for up to 2 hours during device use, with assessments including how easy the device is to use, duration of proper pressure delivery, battery consumption and duration, and changes in blood oxygen levels SpO2. The study will collect data to support the devices future use as a first-aid tool for respiratory failure outside hospitals. The total study duration and follow-up details are not specified.