Clinical Research Directory

Optimizing the Follow-Up Journey in Interstitial Lung Disease: The OPTIMIZE-ILD-2 Trial

The OPTIMIZE-ILD-2 trial is a prospective, randomized, open-label clinical trial designed to evaluate the impact of a coordinated follow-up pathway on patients with established interstitial lung disease (ILD). In routine clinical practice, follow-up workflows for ILD are frequently fragmented, requiring multiple hospital visits for pulmonary function tests, laboratory analysis, treatment administration, and consultations with various specialists, which increases the burden for both patients and caregivers. This study compares the standard follow-up care against an optimized circuit where all routine monitoring procedures and interdisciplinary consultations are pre-bundled and scheduled within a single, coordinated hospital visit. All eligible patients under active ILD follow-up are included consecutively to ensure a pragmatic, real-world representation of the treated ILD population. The primary objective is to measure the total follow-up time burden, defined as the total home-to-home time required to complete the follow-up circuit. As a cross-sectional assessment within a longitudinal context, secondary objectives include assessing socioeconomic cost-burden, the environmental carbon footprint of the follow-up journey, health-related quality of life, and clinical frailty. Caregiver-related outcomes, including burden and experience measures, are contingent upon the presence of a primary caregiver and the provision of their independent informed consent. The design of this protocol was informed by a patient focus group and is officially endorsed by the 'AIRE' Associació Catalana de Malalts i Trasplantats Pulmonars, ensuring a patient-centered approach that prioritizes follow-up efficiency and human impact.

Optimizing the Diagnostic Journey in Interstitial Lung Disease: The OPTIMIZE-ILD-1 Trial

The OPTIMIZE-ILD-1 trial is a prospective, randomized, open-label clinical trial designed to evaluate the impact of a coordinated diagnostic pathway on patients with suspected interstitial lung disease (ILD). In routine clinical practice, diagnostic workflows for ILD are frequently fragmented, involving multiple independent appointments that can lead to significant delays and increased burden for patients and caregivers. This study compares the standard diagnostic pathway against an optimized circuit where core diagnostic procedures-such as high-resolution CT, pulmonary function tests, and laboratory panels-are pre-bundled and scheduled within a coordinated and compressed timeframe. All eligible patients referred for suspected ILD are included consecutively to ensure a pragmatic, real-world representation of the referral population. The primary objective is to measure the time to diagnostic communication, defined as the duration from randomization to the date the patient is formally informed of the final diagnosis following a multidisciplinary team (MDT) consensus. Secondary objectives include assessing the time to MDT diagnosis, the time to treatment initiation (when clinically indicated), socioeconomic cost-burden, and the environmental carbon footprint of the diagnostic journey. Furthermore, the study evaluates health-related quality of life, psychological distress, and clinical frailty, while exploring factors such as language proficiency as determinants of diagnostic equity. Caregiver-related outcomes, including burden and experience measures, are contingent upon the presence of a primary caregiver and the provision of their independent informed consent. The design of this protocol was informed by a patient focus group and is officially endorsed by the 'AIRE' Associació Catalana de Malalts i Trasplantats Pulmonars, ensuring a patient-centered approach that prioritizes the diagnostic journey's efficiency and human impact.

Safety and Efficacy of Nebulized 3D-cultured Human Placental Mesenchymal Stem Cell-derived Extracellular Vesicles (hPMSC-EVs) for Idiopathic Pulmonary Fibrosis

This is a single-center, open-label, dose-escalation clinical study to evaluate the safety and efficacy of nebulized 3D-cultured human placental mesenchymal stem cell-derived extracellular vesicles (hPMSC-EVs) in patients with idiopathic pulmonary fibrosis (IPF). Eligible participants will be assigned to one of three dose groups (1.0×10⁹, 2.0×10⁹, or 3.0×10⁹ particles per treatment) using a 3+3 design. All participants will receive nebulized hPMSC-EVs twice daily for 7 consecutive days. The main purpose of this study is to assess the safety and tolerability of hPMSC-EVs, including the incidence of adverse events, changes in vital signs, laboratory tests, and immune markers. Secondary objectives include evaluating changes in lung function (FVC, DLCO), 6-minute walking distance, respiratory symptoms (SGRQ score), and chest HRCT findings. Participants will undergo screening visits, treatment administration, and follow-up visits up to 12 months after the first dose to monitor safety and efficacy outcomes.

AURA-IPF: A Randomized Phase 2 Study to Evaluate the Safety and Efficacy of AP02 (Nintedanib Solution) in IPF

This study will evaluate the impact Nintedanib Solution for Inhalation (AP02) has on lung function and key measures of fibrosis in adult patients with idiopathic pulmonary fibrosis (IPF) as well as assess its safety and tolerability. Adults 40 years of age or older with IPF who meet the inclusion and exclusion criteria can participate in this study if they are not currently on treatment for IPF, and if treated with oral nintedanib or pirfenidone, have stopped the medication for at least 3 months. Researchers will compare two different doses of AP02 to a placebo (a look-alike substance that contains no drug) to see if AP02 works to treat IPF. Participants are put into 1 of 3 groups randomly, which means by chance and will take AP02 or a placebo two times every day for 12 weeks by using a nebulizer, which is a device that provides medicine to the lungs via inhalation. Participants will visit the office 6 times and receive 1 phone call over a 16-week period. At site visits doctors regularly perform breathing tests that measure how well the lungs are working, give the patient questionnaires and will check the participants' health.

A Phase 2 Study of LTI-03 in Patients With Idiopathic Pulmonary Fibrosis

Rationale: LTI-03 is an experimental medication breathed into the lungs using an inhaler. It is being studied for the treatment of Idiopathic Pulmonary Fibrosis (IPF). IPF is a progressive, fatal lung disease caused by the death of lung cells involved in oxygen uptake and by progressive fibrosis (scarring) of the lungs. As the disease progresses, patients experience loss of lung function and increased breathing problems. LTI-03 is hypothesized to treat IPF by protecting and restoring the function of the oxygen uptake cells and by controlling lung fibrosis which may result in improving lung scarring. The purpose of this research is to evaluate LTI-03 including: its safety, whether it causes side effects, whether it improves lung scarring, and whether it improves IPF symptoms. LTI-03 will be compared to placebo in patients diagnosed with IPF within the last 5 years. Patients on a stable dose of nintedanib, pirfenidone, or nerandomilast (if available by prescription) may participate. Trial Design: This is a Phase 2, randomized, double-blind, placebo-controlled, multi-center study that includes a 28-day Screening Period, a 24-week Treatment Period, and 4-week Follow-up Period. Study Assessments: Up to 9 visits to the study clinic will be required. Safety and tolerability will be evaluated with the following assessments: physical examination; collection of vital sign data (heart rate, blood pressure, respiratory rate and peripheral oxygen saturation \[SpO2\] via pulse oximetry); heart data collected by 12-lead electrocardiogram; and collection of blood samples for safety laboratory tests. In addition, participants will be asked about any adverse events (side effects) they have experienced between clinic visits, if they have changed any medications, and if they are able to properly use their study drug inhaler. Participants will undergo a lung function test (spirometry) at every visit, which will be used to evaluate both safety and efficacy. Another test measuring the diffusion capacity of the lungs for carbon monoxide (DLCO) will be required at Screening only. Blood samples will also be collected at each visit to measure disease biomarkers. At select visits patients will be asked to complete the Living with Pulmonary Fibrosis questionnaire to evaluate their IPF symptoms. Participants will also undergo a specialized lung scan (HRCT) at Baseline and at the End of Treatment to measure changes in lung fibrosis. Interventions: LTI-03 and placebo are provided in powder-filled capsules that participants will self- administer using an inhaler. Placebo capsules look like LTI-03 capsules but have no active ingredients. Approximately 120 participants will be randomly assigned in a blinded manner to one of study drug treatment groups.

Study on the Efficacy of Quercetin Intake in Patients With Fibrotic Interstitial Lung Diseases.

Fibrotic interstitial lung diseases (F-ILDs), including both idiopathic pulmonary fibrosis (IPF) and non-IPF, are chronic and progressive lung diseases characterized by excessive scarring of lung tissue, leading to declining lung function, respiratory failure, and high mortality, despite the currently approved antifibrotic treatment. While its exact cause remains unknown, pulmonary fibrosis is strongly linked to aging, genetic predisposition, environmental factors, and cellular senescence. Ongoing research aims to identify reliable biomarkers and develop targeted treatments to enhance patient outcomes. This randomized controlled trial will examine the effects of quercetin supplementation (500 mg/day for two 12-week cycles, with one 8-week washout periods) on telomere length, senescence-associated secretory phenotype (SASP) factors, and lung function in patients with IPF and F-ILDs. A total of 100 patients will be recruited, with half receiving quercetin (despite their standard of care therapy) and the other half receiving standard care (SOC). Primary outcomes will include changes in telomere length, SASP protein levels (IL-6, MMPs), fractional exhaled nitric oxide (FeNO), spirometry (FVC decline), and oscillometry measurements. Additionally, quality of life will be assessed using the L-IPF Questionnaire. This study aims to explore quercetin's potential to reduce fibrosis, decrease inflammation, and improve lung function in F-ILDs, offering new insights into potential novel strategies for F-ILD management.

A Adaptive Design Clinical Trial to Evaluate the Efficacy and Safety of TDI01 Suspension in the Treatment of Idiopathic Pulmonary Fibrosis (IPF)

This study is a multicentre, randomised, double-blind, placebo-controlled, adaptive design clinical trial to evaluate the efficacy and safety of TDI01 suspension in the treatment of idiopathic pulmonary fibrosis (IPF). The study will be conducted in China and divided into two stages, both of which are multicentre, randomised, double-blind, placebo-controlled studies. Stage 1 aims to evaluate the efficacy and safety of TDI01 suspension compared to the placebo group in the treatment of IPF patients, and Stage 2 aims to further confirm the efficacy and safety of TDI01 suspension compared to the placebo group in the treatment of IPF patients.

A Trial to Evaluate Efficacy and Safety of Buloxibutid in People With Idiopathic Pulmonary Fibrosis.

The ASPIRE trial is a 52 week randomized, double-blind, placebo-controlled, parallel-group, multicenter trial in which the efficacy, safety, and pharmacokinetics of orally administered buloxibutid, either on top of stable IPF therapy or as monotherapy, are assessed in participants with IPF. Trial website: www.aspire-ipf.com

Expand Pulmonary Rehabilitation to Other Chronic Respiratory Diseases Than COPD

The purpose of this pilot randomized clinical trial is to evaluate the willingness, acceptance, adherence and signs of benefits of structured home-based pulmonary rehabilitation (HPR) and supervised pulmonary tele-rehabilitation (PTR) on respiratory symptoms, quality of life, functioning and physical activity in patients with ILD (Interstitial lung disease, Idiopathic pulmonary fibrosis, Sarcoidosis), Bronchiectasis and Asthma who have a clinically assessed need for a pulmonary rehabilitation program. The main questions the project aims to answer are: * Will HPR and PTR appeal to a minimum of 30% of the eligble patients * Be greatly accepted (≥70% of participant complete 70% or more of the planned sessions) by patients agreeing to participate in the study * Will PTR and HPR lead to positive changes in respiratory symptom relief, symptom relief in general and improve or maintain physical functioning larger than usual care Researchers will compare with a comparable control group who gets usual care (scheduled controls and medication but no exercise intervention) to examine the willingness to be randomized and to get an indication on possible health related effects. Participants will be randomly assigned to one of three groups: 1. A structured supervised group-based PTR (exercise twice a week; each session consists of 35 min. evidence-based exercises and 25 min. of patient education) 2. An individual HPR program (self-initiated physical activity) with motivational and professional counseling once a week (10-15min), supported by a tablet screen 3. A control group receiving usual care (CON)

Phase II Clinical Study of BC006 in Patients With Idiopathic Pulmonary Fibrosis

This is a multicenter, Phase II clinical study to evaluate the efficacy and safety of BC006 over a 24-week treatment period in patients with idiopathic pulmonary fibrosis (IPF). The study consists of two phases: an open-label safety run-in phase and a double-blind, randomized, placebo-controlled phase.

An Extension Study of Subjects Who Received an Avalyn Inhaled Antifibrotic Agent (SAIL)

This is an open-label extension study for participants who were previously enrolled in and completed an Avalyn Pharma Sponsored study with an inhaled antifibrotic, such as AP01. Eligible participants will have their final dose of drug at the end of study visit from the lead-in study and first AP-LTE-008 study visit on the same day.

Treatment of Idiopathic Pulmonary Fibrosis (IPF) by REGEND007 Cell Therapy

This study is a prospective, single-arm, dose-escalation exploratory clinical trial to investigate the safety, tolerability and preliminary efficacy of REGEND007 stem cell preparation administered by intravenous infusion in the treatment of idiopathic pulmonary fibrosis (IPF), with a follow-up period of 12 weeks.

A Study to Evaluate Safety, Tolerability and Pharmacokinetics of MNKD-201 in Patients With Idiopathic Pulmonary Fibrosis

MKC-NI-002 is a Phase 1b, randomized, double-blind, placebo-controlled study of nintedanib inhalation powder (MNKD-201) in patients with Idiopathic Pulmonary Fibrosis (IPF). The trial consists of Multiple Ascending Doses (MAD) with the primary objective to evaluate safety, tolerability and pharmacokinetics (PK) of MNKD-201 compared to placebo in patients with IPF.

A Pilot Study to Assess Body Mass Composition Measurement Using BIA and Muscle Ultrasound in IPF and PPF Patients on Anti-fibrotic Medications

To assess the impact on body mass composition from anti-fibrotic medications used in fibrotic lung disease by using BIA and muscle ultrasound

Trial to Evaluate the Efficacy and Safety of LYT-100 (Deupirfenidone) Compared to Pirfenidone in Adults With Idiopathic Pulmonary Fibrosis (IPF)

This is a study for adults with a lung disease called idiopathic pulmonary fibrosis. The main purpose of this study is to look at how well deupirfenidone improves lung function and how safe it is for people with idiopathic pulmonary fibrosis (IPF) when compared with pirfenidone. Participants may have been treated with an approved antifibrotic drug for up to a year in the past, but they cannot be on background antifibrotic treatment during this study. Participants will be randomly assigned (meaning by chance) to take either deupirfenidone or pirfenidone 3 times a day, and neither a participant nor their study team will know which study drug participants are on. Participants will be in the study for up to approximately 3 years. During the first year, participants visit the study site up to ten times and afterwards they visit the site every three months. All participants will remain on blinded study drug until the last participant has completed Week 52 Visit. They will have lung function tests, a check of their health, and will tell the study team about any unfavorable effects.

SB17170 Phase 2 Trial in IPF Patients

This clinical trial is a 2:2:1 randomized, double-blind, placebo-controlled, parallel group, exploratory phase II trial. The main objective of this trial is to compare and evaluate change in FVC compared to placebo by administering SB17170 to moderate to severe patients with IPF. This clinical trial treatment involves administering SB17170 or placebo for 12 weeks.

Collection of Airway, Blood and/or Urine Specimens From Subjects for Research Studies

The purpose of this study is to obtain biologic materials from the blood, airways and/or urine of normal individuals and individuals with lung disease. The normal are used to establish a set of normal ranges for various parameters. These provide control information when compared to individuals with various pulmonary diseases, and will help in understanding of the etiology and pathogenesis of various lung diseases. The underlying hypothesis is that the pathologic morphological changes in the airway epithelium must be preceded by changes in the gene expression pattern of the airway epithelium and potentially in macrophages.

Study Evaluating INS018_055 Administered Orally to Subjects With Idiopathic Pulmonary Fibrosis

The purpose of this revised Phase IIa study is to demonstrate safety of INS018\_055 over 12 weeks in adults with Idiopathic Pulmonary Fibrosis (IPF).

Motivational Interviewing Program on the Self-Care in Idiopathic Pulmonary Fibrosis

A specific tool developed to measure self-care in patients with Idiopathic Pulmonary Fibrosis (IPF) is the SC-IPFI, which is an adaptation of an already validated instrument for COPD. It is important to assess how motivational interviewing can influence the self-care behaviors of these patients, better evaluate their level of disease management, prevent the risk of exacerbations, and guide educational interventions. The primary objective of this study is to evaluate the effectiveness of a motivational interviewing program delivered remotely through video calls and digital platforms in improving the levels of self-care maintenance, self-care monitoring, and self-care management in patients with IPF. The study will also assess the effectiveness of the remotely delivered motivational interviewing program on self-efficacy, anxiety, disease-specific quality of life, therapy adherence, cough, dyspnea, exacerbations, and hospital admissions.

Disease-syndrome Characteristics of IPF

This study, based on previous registration records, aims to explore the syndrome patterns, disease characteristics, and their interrelationships of IPF at different stages, grades, and in its natural course, thus providing a multidimensional interpretation of the syndrome patterns and characteristics of IPF.

Radiologic Features of Rheumatoid Arthritis Interstitial Lung Disease at Chest High Resolution Computed Tomography

Lung involvement is one of the most frequent extra articular involvements of rheumatoid arthritis (RA). RA related lung involvement can affect parenchyma, airway, vascular tree and serosa. Among all possible manifestations of lung disease, interstitial lung disease is the most severe being able to compromise quality of life and survival and involves about 20% of patients with rheumatoid arthritis (RA). However, chest high resolution computed tomography (HRCT) features of RA, with or without interstitial lung involvement, have not been clearly defined. Such features have been mostly investigated on small populations of RA patients, often including patients with connective tissue diseases (CTDs), namely systemic sclerosis, mixed connective tissue disease, idiopathic inflammatory myopathies, making difficult to discriminate possible specific features of RA interstitial lung disease (ILD). The aims of this observational longitudinal study are to investigate: i) chest HRCT features of RA (frequency of radiologic HRCT patterns, fibrosis, nodules, bronchiectasis, etc.), associated or not to ILD; ii) possible associations between chest HRCT features and demographic, clinical and serologic characteristics of RA; iii) specific chest HRCT features of RA ILD, compared to idiopathic pulmonary fibrosis (IPF) and CTD ILD (i.e., primary Sjogren syndrome, idiopathic inflammatory myopathies, etc), according to the Centres availability. Consecutive, unselected, DICOM files of chest HRCT of adult RA patients (regardless a previous diagnosis of ILD) will be evaluated by an expert thoracic radiologist blinded to patients' clinical history. HRCT patterns, presence of fibrosis and other lung abnormalities (cysts, nodules, pleural effusion, etc) will be recorded. In patients with RA ILD possible associations with demographic and clinical disease features will be also analysed (such as sex, disease duration, disease duration at ILD diagnosis, presence of ACPA, rheumatoid factor, ANA), inclusion of previous therapies. After 2 years, new HRCT and lung function tests will be collected for each enrolled patients when available, to evaluate possible changes of lung involvement over time.

Idiopathic Pulmonary Fibrosis Cohort of TCM

This study, based on past registration records, evaluates the clinical efficacy of traditional Chinese medicine in treating IPF in the real world, identifies advantageous populations and clinical targets, and provides references for precision traditional Chinese medicine treatment.

Menstrual Blood-Derived Mesenchymal Stem Cell Injection (SC01009) in the Treatment of Idiopathic Pulmonary Fibrosis

At present, the diagnosis of IPF is still difficult and it cannot be completely cured. Treatment is still aimed at improving symptoms and delaying the decline of lung function. The treatment measures for IPF mainly include drug therapy ( pirfenidone and nintedanib), non-drug therapy (such as oxygen therapy, pulmonary rehabilitation), treatment of complications/comorbidities, symptomatic treatment and palliative treatment. However, IPF cannot be completely cured at present, so lung transplantation is a feasible option for the treatment of IPF. However, due to the scarcity of donor lungs, prevention of acute and chronic rejection reactions, post-transplant infection complications, and high costs, its clinical implementation is limited. Therefore, there is still a large unmet treatment need for IPF, and new therapeutic drugs are urgently needed. In recent years, mesenchymal stem cells (MSCs) have been considered a new hope for the treatment of IPF. MSCs are a type of multipotent stem cells with the potential for self-proliferation and differentiation. They have the characteristics of self-renewal, multidirectional differentiation, low immunogenicity, and paracrine. They can automatically home to damaged areas, promote epithelial tissue repair, inhibit inflammation, and inhibit abnormal proliferation of fibroblasts . Many clinical trials at home and abroad are exploring the effectiveness and safety of MSC in the treatment of pulmonary fibrosis. An Australian study found that in patients with moderate to severe IPF, intravenous infusion of umbilical cord MSCs up to 2 × 106 cells/kg was safe, and no decrease in forced vital capacity (FVC), diffusing vital capacity for carbon monoxide (DLCO), 6-minute walk test distance (6MWD), and CT fibrosis score was observed during a 6-month follow-up . The results of the American AETHER study also showed that a single intravenous infusion of up to 2 × 10\^ 8 cells of bone marrow MSCs was safe, with no serious treatment-related adverse events observed. Moreover, 60 weeks after infusion , FVC% decreased by 3.0% and DLCO% decreased by 5.4%, both of which were below the threshold for disease progression . This study adopted a multicenter, randomized, double-blind, placebo-controlled, parallel design to evaluate the efficacy and safety of SC01009 injection in IPF subjects . This study plans to enroll 66 subjects, who will be randomly stratified according to whether they have received oral pirfenidone/nintedanib treatment at screening , and randomly assigned to two trial groups and one placebo group in a 1:1:1 ratio. This study includes a screening period of up to 4 weeks, a 24-week treatment follow-up period, and a 2-year long-term follow-up period. After screening, qualified subjects will be given medication according to the following dosing schedule according to their group: Trial Group 1: received intravenous infusion of SC01009 injection in week 1, divided into 3 infusions every other day, each infusion of 3 × 10\^ 7 cells, with a total dose of 9 × 10 \^7 cells; received intravenous infusion of placebo in week 13, with the same administration method as in week 1; Experimental group 2: received intravenous infusion of SC01009 injection at week 1 and week 13, respectively. Each administration was divided into 3 infusions every other day, with 3 × 10\^7 cells in each infusion , and the total dose was 1.8 × 10\^8 cells. Placebo group: received intravenous infusion of placebo at week 1 and week 13, respectively, with the same administration method as the cell therapy group. After completing 24 weeks of treatment follow-up, all subjects entered the long-term follow-up phase and were unblinded after the study database was locked . The subjects in the original placebo group could end the follow-up, while the subjects receiving cell therapy would continue to be followed up for up to 2 years.

Confirmatory Clinical Study of HEC585 Tablets in Patients With IPF

A multicenter, parallel, randomized, placebo (double-blind) and pirfenidone (open-label) controlled Phase III clinical trial to evaluate the efficacy and safety of HEC585 in patients with idiopathic pulmonary fibrosis (IPF)