Interstitial Lung Disease

Researchers are evaluating new imaging techniques in adults aged 18 to 85 with lung diseases such as asthma, emphysema, COPD, bronchiectasis, sarcoidosis, pulmonary fibrosis, alpha 1-anti-trypsin deficiency, and lymphangioleiomyomatosis (LAM). The study aims to develop tools for analyzing lung function and structure using hyperpolarized Xenon 129 MRI alongside pulmonary function tests. This research is supported by Western University, Canada. Participants will undergo a series of tests during a one to two-hour visit, including medical history review, vital signs measurement, full pulmonary function testing following American Thoracic Society guidelines, proton MRI, specialized 129-Xe MRI scans using chest coils, and a low-dose thoracic CT scan. The MRI procedure includes monitoring heart rate and oxygen levels, providing hearing protection, and offering supplemental oxygen as needed. These imaging and testing methods help assess lung ventilation, diffusion, and gas exchange. During the study visit, participants will complete pulmonary function tests such as spirometry, plethysmography, and diffusing capacity tests. They will undergo magnetic resonance imaging with hyperpolarized Xenon gas to visualize lung airways and anatomy, along with CT scans matched to MRI breathing maneuvers. Researchers will measure ventilation defect percent (VDP), apparent diffusion coefficients (ADC), and dissolved phase spectroscopy over five years. Participant safety is monitored throughout the visit, and the total study duration includes these assessments and follow-up measures.

Researchers are studying biomarkers from 129Xe gas exchange MRI to understand how they change with different medical interventions. The study focuses on interactions between 129Xe and red blood cells in the lungs. Participants include healthy volunteers and patients with conditions like interstitial lung disease, pulmonary hypertension, acute or chronic pulmonary embolism, anemia, polycythemia, and dyspnea. The study uses hyperpolarized xenon gas inhaled in multiple doses followed by breath holds, alongside oxygen administration. Participants are grouped by treatment: those undergoing transfusion or phlebotomy, patients receiving oxygen for lung-related conditions or healthy volunteers, and those recently diagnosed with acute or chronic pulmonary embolism. Treatments and responses are monitored at baseline and various follow-up points. Participants will undergo MRI scans to measure red blood cell transfer, chemical shifts after oxygen delivery, and changes in red blood cell signal oscillations before and after treatment. The study includes visits up to 3–6 months after interventions to track changes. Researchers also monitor safety and participant adherence throughout the study, which lasts until mid-2028.

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.

Researchers are conducting the GENESIS clinical study to map the HLA genomic region in the Greek population and explore its possible links with various underlying diseases. This non-interventional, multicenter study aims to provide a pilot map of genetic variation in HLA that may be useful in medical research and clinical applications related to selected diseases. The study plans to include 12,000 participants over a total duration of 36 months. Each participant will attend one visit at a participating site during which they will provide demographic data, lifestyle information such as smoking and alcohol use, blood pressure measurements, details on diagnosed diseases and treatments, and recent laboratory test results if available. Buccal swab samples will be collected from each participant to extract DNA for HLA genotyping analysis. Selected samples will undergo further whole genome sequencing to investigate associations with autoimmune diseases. Participants will receive a personalized ancestry report after analysis completion. During the study visit, data collection includes demographic and health information, as well as laboratory and clinical test results from the past year. The genetic material from buccal swabs will be stored and processed for genetic analysis. Researchers will measure allele frequency of HLA alleles in the Greek population and assess the prevalence and risk associations of selected HLA-related diseases. The study's total duration is 36 months with results available at the end of this period.

Researchers are studying people with idiopathic pulmonary fibrosis (IPF) or progressive pulmonary fibrosis (PPF) who have previously completed treatment with the drug nerandomilast. The study aims to understand how well participants tolerate long-term treatment with nerandomilast and whether it helps improve lung function or delays worsening symptoms, hospitalizations, or death. This is a phase 3 open-label extension trial sponsored by Boehringer Ingelheim. Participants will take nerandomilast tablets daily for up to 1 year and 10 months. They may continue their usual treatments for pulmonary fibrosis alongside the study treatment. There is no placebo group, and all participants receive nerandomilast during this extension period. Throughout the study, participants will visit their doctors regularly for health check-ups and lung function tests. Researchers will closely monitor any side effects and changes in lung function, particularly forced vital capacity (FVC), over nearly two years. The study will track time until symptom worsening, hospital visits, or death to assess the treatment's long-term safety and effects.

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 nodules/masses, transbronchial needle aspiration biopsy of peripheral lung nodules/masses, transbronchial needle aspiration biopsy of mediastinal/hilar lymph nodes, and transbronchial cryo biopsy of peripheral lung nodules/masses. 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 technology's 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 Organization's 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.