Idiopathic Pulmonary Fibrosis

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 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 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.

Coughing is a common and distressing symptom for people with interstitial lung disease (ILD), affecting daily activities, work, and social life. This research explores a non-drug cough control therapy combined with pulmonary rehabilitation to see if it is practical and helps reduce chronic cough lasting more than eight weeks in ILD patients. The study aims to assess how well patients accept this therapy and whether it improves quality of life, cough symptoms, and fatigue compared to standard pulmonary rehabilitation alone. Participants will join a pulmonary rehabilitation program involving aerobic and strengthening exercises, disease-specific education, and self-management as usual care. Two weeks before finishing this program, they will receive four virtual sessions of 45 to 60 minutes each, focusing on cough control education and techniques. These sessions include assessing cough, learning suppression methods, breathing retraining, and reinforcing strategies to manage cough in daily life. During the study, researchers will track enrollment and attendance to measure feasibility, along with various clinical outcomes like cough severity questionnaires, fatigue scales, breathlessness ratings, and patient satisfaction interviews. The intervention is delivered online via Zoom, allowing remote participation. The total study duration averages one year, with ongoing assessments to understand the therapy's impact and participants' experiences.

Researchers are studying HW241045 in a Phase Ia clinical trial to evaluate its safety, tolerability, and how it is processed in the body after a single dose. This randomized, double-blind, placebo-controlled study involves healthy adult volunteers aged 18 to 55 years to better understand the effects of this drug compared to a placebo. Participants will receive a single oral dose of either HW241045 or a placebo. The study is designed with two groups: one receiving the experimental drug and the other receiving placebo, both under blinded conditions to ensure unbiased results. The trial is conducted at a single center and uses a quadruple masking approach. During the study, participants will be closely monitored for any treatment-emergent adverse events within 48 hours after dosing. Assessments include safety checks, tolerability evaluations, and pharmacokinetic measurements. Participants' health will be carefully observed during this period to understand the drug’s effects and side effects. The total duration of participation is determined by these assessments and monitoring schedules.

Researchers are evaluating a digital self-management package called REBUILD-SM designed for people with interstitial lung disease (ILD). The study aims to find out whether REBUILD-SM can improve health-related quality of life, symptoms, anxiety, self-confidence in managing illness, and physical activity levels compared to standard care. REBUILD-SM combines a smartphone app with a self-management program developed in collaboration with people living with ILD to support better disease management. Participants in the intervention group will use the REBUILD app to enter and track health data such as diagnosis, medications, lung function, and oxygen use. They will also work through 13 self-management modules at their own pace with support from healthcare professionals through four phone or Zoom calls over a 12-week period. The control group will use a simpler version of the app for data entry only and receive calls without health advice. After 26 weeks, control participants will gain access to the full app but not the self-management content or support calls. During the study, participants will complete questionnaires and update their health data at baseline, 12, 26, and 52 weeks. Electronic reminders and follow-up calls will encourage questionnaire completion. Researchers will measure changes using questionnaires on lung disease impact, quality of life, anxiety, physical activity, and self-efficacy. The main outcome is change in the King's Brief Interstitial Lung Disease questionnaire score at 12 weeks. The study includes ongoing monitoring up to 52 weeks to assess longer-term effects and app usability.

This research evaluates the safety, tolerability, pharmacokinetics, and pharmacodynamics of GNS-3545 in healthy adult volunteers aged 18 to 55. It is a randomized, double-blind, placebo-controlled Phase 1 study that includes single ascending dose (SAD) and multiple ascending dose (MAD) parts, with an additional assessment of the food effect on the drug in one group during Part 1. Participants in Part 1 will receive a single oral dose of GNS-3545 or matching placebo, while in Part 2, participants will take daily oral doses for 14 consecutive days. Each cohort includes six participants receiving GNS-3545 and two receiving placebo. The study monitors how the drug is processed in the body and its effects after single and multiple doses. During the study, participants will be closely observed for any treatment-emergent adverse events from dosing through follow-up periods. Pharmacokinetic measurements such as drug concentration over time, peak levels, and urine excretion will be collected at specified intervals. The total participation involves dosing days and additional monitoring days up to 17 days post-dosing, ensuring thorough safety and drug behavior assessment.

Researchers are evaluating the safety and effectiveness of two doses of inhaled pirfenidone (AP01) compared to a placebo, alongside standard care, in people with progressive pulmonary fibrosis (PPF) over a 52-week period. This randomized, double-blind, placebo-controlled Phase 2b study aims to better understand treatment options for individuals with PPF, a condition characterized by worsening lung fibrosis despite existing therapies. Participants will be randomly assigned to one of three groups: a high dose of AP01 twice daily, a low dose of AP01 twice daily, or a placebo inhalation twice daily. The study involves taking inhaled treatments using a pirfenidone solution or placebo, with the goal to evaluate the impact of these treatments on lung function and disease progression over one year. Throughout the study, participants will undergo regular assessments including lung function tests, imaging scans, and quality of life evaluations. Researchers will monitor the effect of the treatments on lung fibrosis, respiratory symptoms, and safety measures. The total participation lasts for 52 weeks, during which participants will attend scheduled visits and complete various tests to track their health and response to the study treatments.

Researchers are studying adults aged 40 years and older who have idiopathic pulmonary fibrosis (IPF) to find out whether the medicine pirfenidone affects the levels of another medicine called BI 1015550 in the blood. The study aims to understand how these two medicines interact when taken together, which is important since patients may take more than one medicine at a time. This is a Phase 2 trial sponsored by Boehringer Ingelheim. Participants first take a single dose of BI 1015550 as a tablet. Then, they start taking pirfenidone tablets three times a day, starting with one tablet per dose during the first week, increasing to two tablets per dose in the second week, and three tablets per dose in the third week. After this dose escalation, participants take another single dose of BI 1015550. The study is open-label and involves one treatment group. Participants will be involved in the study for a little over one month, during which they will visit the study site 15 times. Two of these visits include overnight stays. Researchers will collect health information and take blood samples to measure the amounts of pirfenidone and BI 1015550 in the blood. Throughout the study, participants' health is regularly checked, and any side effects are carefully recorded. The main outcomes measured are the concentration levels of BI 1015550 in the plasma over time.

Researchers are evaluating the safety and tolerability of inhaled mosliciguat combined with inhaled treprostinil in adults with pulmonary hypertension associated with interstitial lung disease (PH-ILD). This Phase 2, open-label, multi-center study focuses on participants who already use inhaled treprostinil to manage their condition. The trial aims to gather important safety information about this combination treatment in this specific lung disease population. Participants will receive daily inhaled mosliciguat during a 16-week open-label treatment period. After this period, those who complete the initial phase may continue to receive mosliciguat in an extension phase lasting beyond 16 weeks. Mosliciguat will be delivered using a dry powder inhaler device, and doses may vary among three different levels. All participants will continue treatment with inhaled mosliciguat during the extension. Throughout the study, researchers will monitor participants for treatment-emergent adverse events, serious adverse events, and any side effects leading to discontinuation, with assessments at baseline and at week 16. Participants must be able to perform a six-minute walk of at least 100 meters. The study includes imaging confirmation of interstitial lung disease and right heart catheterization to confirm pulmonary hypertension. The total participation duration may extend beyond 16 weeks depending on continuation in the extension phase.