Clinical Research Directory

Long-term Extension Study to Evaluate Safety and Tolerability of Admilparant in Participants With Pulmonary Fibrosis

The purpose of this study is to evaluate the long-term safety and tolerability of Admilparant in participants who completed participation in parent studies IM027-068 (for idiopathic pulmonary fibrosis (IPF)) and IM027-1015 (for progressive pulmonary fibrosis (PPF)).

Role of Genetic Factors in the Development of Lung Disease

This study is designed to evaluate the genetics involved in the development of lung disease by surveying genes involved in the process of breathing and examining the genes in lung cells of patients with lung disease. The study will focus on defining the distribution of abnormal genes responsible for processes directly involved in different diseases affecting the lungs of patients and healthy volunteers. Optional CT Sub-study The standard CT scan will be compared to the low dose radiation CT scan for the 150 subjects enrolled in the sub-study to assess the variation between the two techniques. Specifically, the quantitative computer aided detection of lung CT abnormalities from LAM can be compared to assess whether low radiation dose CT exams is an alternative to conventional CT to monitor disease status. This optional sub-study will be offered to up to 100 adult subjects with lung disease and up to 50 children age 9 and older with CF. Children will not be enrolled in the optional CT sub-study unless they have had a standard CT scan for medical purposes to use in comparison. One additional low dose radiation CT scan of the chest may be done as part of this sub-study when these subjects have their next annual CT scan.

Analysis of Specimens From Individuals With Pulmonary Fibrosis

The etiology of pulmonary fibrosis is unknown. Analyses of blood, genomic DNA, and specimens procured by bronchoscopy, lung biopsy, lung transplantation, clinically-indicated extra-pulmonary biopsies, or post-mortem examination from individuals with this disorder may contribute to our understanding of the pathogenic mechanisms of pulmonary fibrosis. The purpose of this protocol is to procure and analyze blood, genomic DNA, and specimens by bronchoscopy, lung biopsy, lung transplantation, extra-pulmonary biopsies, or post-mortem examination from subjects with pulmonary fibrosis. In addition, blood, genomic DNA, clinically-indicated extra-pulmonary biopsies, as well as bronchoscopy and post-mortem examination specimens may be procured and analyzed from relatives of subjects with hereditary forms of pulmonary fibrosis; blood, genomic DNA, and bronchoscopy specimens may be procured from healthy research volunteers.

Role of the Fibroblast Activation Protein (FAP) as Biomarker of Fibrotic Lung Diseases

To evaluate the effect of an anti-fibrotic treatment initiation on the fibrotic activity as assessed by FAPI PET/CT.

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.

Telomeres Length in Israeli Fibrotic ILD Patients

Individuals with fibrotic interstitial lung diseases (FILD) will be recruited after providing informed consent. in addition to routine data as usually collected in the clinic, blood samples will be taken for measurement of telomeres length in peripheral blood leukocytes using both the Telomere Restriction Fragment (TRF) Analysis method and Nanopore sequencing. Participants with FILD will be followed-up for 2-year after recruitment, including clinical and pulmonary function tests at-least every 6 months, or more frequently, according to the treating physician discretion.

Comparison of Pulmonary Telerehabilitation and Physical Activity Recommendations in Patients With Post Covid Fibrosis

Rehabilitation plays a very important role in the management of patients with COVID-19, focusing on respiratory and motor functions, and therefore the importance of establishing treatment strategies to ensure optimal recovery of these patients has been emphasized. It has been stated that physical activity recommendations should be clarified for the management of symptoms associated with prolonged COVID-19 Syndrome and for the continuation of activities of daily living. It has been stated that after COVID-19 pneumonia, it is necessary to evaluate the physical functions of patients with long-term follow-up and to establish rehabilitation programs. The importance of being included in the rehabilitation program was emphasized, especially for patients with lung fibrosis. The primary aim of this study was to compare the effects of pulmonary telerehabilitation and physical activity recommendations on exercise capacity and peripheral muscle strength in patients with pulmonary fibrosis due to COVID-19. The secondary aim of this study is to compare the effects of pulmonary telerehabilitation and physical activity recommendations on symptoms, activity and participation in patients with pulmonary fibrosis due to COVID-19.

Study on the Drug Interactions of HRS-9813, Pirfenidone and Nintedanib in Healthy Subjects

This study aims to evaluate the interaction of oral HRS-9813 capsules with pirfenidone and nintedanib on the pharmacokinetics of healthy subjects.

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

Post Acute Sequelae of COVID-19

COVID-19, a novel coronavirus, has caused widespread mortality and morbidity since it emerged in 2019. There is ongoing research and growing literature describing severe acute respiratory syndrome (SARS-COV-2). There is a growing population of individuals who have recovered from acute SARS-COV-2 infection. The long-term effects of COVID-19 are unknown. There are growing reports of sequelae after acute SARS-CoV-2 not limited to fatigue, dyspnea, reactive airway disease, organizing pneumonia, pulmonary fibrosis, pulmonary hypertension, pulmonary emboli, and tracheal disease. The incidence and natural history of these findings is unstudied.

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.

The BALANCE Study: A Study in Spain to Find Out Whether a Patient Support Program Helps People With Pulmonary Fibrosis Who Take Nintedanib

The aim of this study is to describe patients' satisfaction with Patient Support Program (Balance Program), Quality of Life and depression symptoms, dosing pattern, disease symptoms, adverse events and nintedanib discontinuation (both permanent and non-permanent) from study inclusion to 12 months of follow-up.

Diaphragmatic Release vs Inspiratory Muscle Training in Pulmonary Fibrosis

This study evaluates the efficacy of adding manual Diaphragmatic Release Technique (DRT) versus Targeted Inspiratory Muscle Training (IMT) to standard medical therapy in patients with stable pulmonary fibrosis. Forty participants are randomly allocated into two equal groups: Group A: Receives manual therapy (cranial and lateral traction to the rib cage for 10-15 minutes) 3 times per week for 12 weeks alongside standard medical care. Group B: Performs targeted inspiratory muscle training using a threshold device (5-7 sets of 6-10 breaths at 30-40% perceived effort) 3 times per week for 12 weeks alongside standard medical care.

Microarray Analysis of Gene Expression in Idiopathic Pulmonary Fibrosis (IPF)

This study is investigating the way the lung is damaged in a condition called pulmonary fibrosis. Research studies will be conducted on lung tissue obtained from an open lung biopsy performed by the subject's surgeon. The identification of unique genetic markers of scarred lung may ultimately lead to new approaches to the diagnosis and treatment of pulmonary fibrosis.

Genetic Polymorphisms in Idiopathic Pulmonary Fibrosis (IPF)

The purposes of this study are: * to determine if there are specific genetic traits that might explain why patients have developed pulmonary fibrosis; * to determine if specific genetic traits account for differing patterns of inflammation and scar tissue that has formed in the patient's lungs.

Feasibility Randomised Controlled Trial Exploring Implementation of the Compassionate Mindful rEsilience pRogram for People With pUlmonaRY Fibrosis: The MERcURY Study

Pulmonary fibrosis (PF) is a chronic lung condition in which scar tissue gradually forms, impairing oxygen transfer and causing breathlessness. Individuals with PF frequently experience anxiety and depression, yet supportive care remains underdeveloped, and few evidence-based psychological interventions exist for this population, representing a critical gap in care. This feasibility randomised controlled trial (RCT) evaluated the Compassionate Mindful Resilience (CMR) programme, a 4-week online, nurse-led mindfulness-based intervention designed to improve psychological outcomes in people living with PF.

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.

Retrospective Validation of AccuPulmo CT Portal for Detecting Pulmonary Fibrosis on Chest CT

This retrospective observational study evaluates the diagnostic performance of AccuPulmo CT Portal, an artificial intelligence-assisted medical imaging software, for detecting pulmonary fibrosis on pre-existing chest computed tomography images. A total of 900 chest computed tomography examinations obtained at Taichung Veterans General Hospital between January 1, 2020, and December 31, 2024, will be retrospectively selected. The planned sample includes 300 examinations with pulmonary fibrosis and 600 examinations without pulmonary fibrosis. All study images will be de-identified and coded before evaluation. Three qualified specialists in pulmonology or radiology will independently review each image without access to the original radiology report or the artificial intelligence output. The reference standard will be established by majority agreement of at least two of the three specialists. AccuPulmo CT Portal will retrospectively analyze the coded images. An artificial intelligence-derived pulmonary fibrosis area greater than 10 percent will be classified as positive, and an area of 10 percent or less will be classified as negative. The primary performance measures are sensitivity and specificity. Secondary measures include accuracy, positive predictive value, negative predictive value, and performance across clinically relevant subgroups. The software results will not be returned to treating physicians and will not affect participant diagnosis, treatment, or clinical management.

Antifibrotic Therapy Decision-Making in Pulmonary Fibrosis

This study will explore how adults in Japan with pulmonary fibrosis decide whether to continue or discontinue antifibrotic therapy after experiencing antifibrotic therapy-related adverse events.

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).

Interstitial Lung Disease Research Unit Biobank

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

Single Time Point Prediction as Earlier Diagnosis of Progressive Pulmonary Fibrosis

This study is a prospective observational study for subjects with idiopathic pulmonary fibrosis (IPF) or non-IPF interstitial lung diseases (ILD). The purpose of this study is to compare whether imaging patterns from high-resolution computed tomography (HRCT) at baseline can predict worsening. Single Time point Prediction (STP) is a score derived from an artificial intelligenc/ machine learning (AI/ML) using the radiomic features from a HRCT scan that quantifies the imaging patterns of short-term predictive worsening.

Safety of Bronchoscopy in Patients With Interstitial Lung Disease

Bronchoscopy is an essential technique, routinely used in the differential diagnosis workup of many Interstitial Lung Diseases (ILDs) and in referral centres is a common procedure. Bronchoalveolar lavage (BAL), bronchial and transbronchial biopsies with forceps and cryoprobes, and lymph node sampling with endosonography represent the most used sampling techniques in these patients. However, patients with ILDs refer to medical attention in a wide range of clinical conditions from mild functional impairment, with absent or few respiratory symptoms, to severe lung involvement with low exercise tolerance and/or chronic respiratory failure. In these patients, a balance between benefits and risk, i.e. to the safety and diagnostic utility of bronchoscopy should be always carefully evaluated. Moreover, there is a wide variability in adverse events entity and frequency depending by procedures performed during bronchoscopy. Despite its crucial utility, only few data are available in the literature on the safety of bronchoscopy in patients with ILDs and limited data on the utility and safety of this sampling technique are present in patients with AE-ILDs. The primary aim of this study is to assess the overall rate of complications occurring within 24 hours after bronchoscopy. Study rate and type of complications occurring during the endoscopic procedure, within 30 days after bronchoscopy, in patients with AE-ILDs, among fibrotic Vs non-fibrotic ILD, and according to each employed sampling technique will be also recorded.

Implementation of Home Monitoring in Patients With Pulmonary Fibrosis

The objective of this study is to evaluate the impact of structurally replacing half of the outpatient clinic visits for patients with pulmonary fibrosis by home monitoring and video consultations on patient self-management and health(care) outcomes.