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.

Multi-site Study of the Clinical Impact of an AI-assisted Approach to Referring Patients With Interstitial Lung Disease for Diagnostic Evaluation of Pulmonary Hypertension

MOMENTOUS is a multi-center, randomized study to prospectively evaluate the performance of an ECG-based AI device to predict whether participants with interstitial lung disease (ILD) are at high risk of undiagnosed pulmonary hypertension.

Robotic-Assisted Versus Conventional Bronchoscopy for Cryobiopsy in the Diagnosis of Interstitial Lung Disease

Interstitial lung disease (ILD) often requires histopathological confirmation when high-resolution computed tomography (HRCT) findings are inconclusive. Transbronchial lung cryobiopsy has emerged as a less invasive alternative to surgical lung biopsy, but diagnostic yield remains variable and dependent on biopsy location and procedural precision. Robotic-assisted bronchoscopy (RAB) combined with cone-beam computed tomography (CBCT) may allow more precise navigation to target lung segments and potentially improve biopsy quality and diagnostic yield. This multi-center, investigator-initiated randomized controlled trial will compare conventional bronchoscopy with 2D fluoroscopy guidance versus robotic-assisted bronchoscopy with CBCT guidance for transbronchial lung cryobiopsy in patients undergoing diagnostic evaluation for suspected interstitial lung disease. Participants will be randomized in a 1:1 ratio to undergo either conventional bronchoscopy or robotic-assisted bronchoscopy. In both groups, four cryobiopsies will be obtained from two lung lobes using a 1.7 mm cryoprobe (en-bloc removal for both study arms). The primary endpoint is the rate of definitive histological diagnosis as determined by a blinded multidisciplinary ILD board.

Treatment in Patients With Advanced Non-Small Cell Lung Carcinoma and Interstitial Lung Disease

Lung cancer is a leading cause of cancer-related death worldwide. Interstitial Lung Diseases are closely associated with lung cancer either as complications or comorbidities to be considered for treatment. Recently, a survey concerning the management of lung cancer in patients with ILDs was conducted by the Interstitial Lung Diseases and Thoracic Oncology Assemblies of the European Respiratory Society. Out of 494 practitioners, mostly pulmonologists, this survey showed that the majority of metastatic patients with pulmonary fibrosis would not be treated (69%), but that 25% and 31% of clinicians would offer chemotherapy or immunotherapy, respectively. The systemic therapy is not clearly codified. There is a risk of worsening of ILDs with most of the treatments used in lung cancer including surgery, radiation therapy or certain systemic therapies. The Japanese Society of Pneumology has recently published proposals for care. However, the Asian population is unique in its incidence of ILDs and the frequency of drug toxicities and these recommendations may not be relevant for other populations. Thus, data are still needed to validate carboplatin and weekly paclitaxel as the best regimen for first-line treatment of NSCLC patients with ILD in a caucasian population. In 2nd line setting, immune checkpoint blocker (ICB) in monotherapy or associated with chemotherapy has become an essential part of the therapeutic arsenal in advanced NSCLC. Several agents have been shown to be superior to docetaxel, following platinum-based chemotherapy failure, and have resulted in several marketing authorizations for PD-1 inhibitors (nivolumab, pembrolizumab) and PD-L1 inhibitors (atezolizumab). We now have the long-term benefits of using ICBs as second-line therapy. Survival at 5 years is 10% higher than that obtained with docetaxel alone. The safety profile is well known in particular with a risk of pulmonary toxicity. It should be noted that in most trials, patients with ILDs were not included. Therefore, we do not have trial data from these pivotal trials in patients with concomitant ILD. Two prospective studies are available on the use of nivolumab in the second-line setting in patients with idiopathic ILDs. The first, in an Asian population, included 6 patients. It showed an interesting response rate of 50% without grade III or IV pulmonary toxicity or worsening of at 12 weeks. Following this, the same team proposed a multicenter phase 2 study. Included patients had mild ILDs (VCf \>80%) and were treated with nivolumab in 2nd line. The primary objective was PFS at 6 months. 18 patients were treated. 3 patients developed toxicity leading to discontinuation of nivolumab including 2 patients with grade 2 pneumonitis. PFS at 6 months was 56%, response rate was 39% and disease control achieved for 72% of patients. In a recent prospective study in Asia, atezolizumab was administered to patients with moderate IPF and advanced NSCLC. The study was stopped prematurely due to a high incidence of inflammatory pneumonitis. Thus, data are still needed to assess the safety of ICB in NSCLC patients with ILD in second line setting.

Effects of Clinical Pilates on Physical and Psychosocial Outcomes in Individuals With Interstitial Lung Disease

This study aims to investigate the effects of Clinical Pilates on physical and psychosocial outcomes in individuals with interstitial lung disease (ILD). Interstitial lung disease is a group of chronic conditions that affect lung tissue and can lead to breathing difficulties, reduced physical capacity, fatigue, and decreased quality of life. In addition to medical treatment, exercise-based rehabilitation approaches may help individuals with ILD improve their physical function and overall well-being. Clinical Pilates is a structured exercise method that focuses on breathing control, posture, core stability, flexibility, and body awareness. Participants in this study will be randomly assigned to either a Clinical Pilates group or a control group receiving standard care. The Clinical Pilates program will be delivered by a trained physiotherapist over several weeks through supervised sessions. The main outcomes of this study include physical function, respiratory symptoms, quality of life, and psychological well-being. The results of this study are expected to provide evidence on whether Clinical Pilates can be an effective and safe supportive approach for pulmonary rehabilitation in individuals with interstitial lung disease.

A Study on the Efficacy and Safety of JAK Inhibitors Versus Calcineurin Inhibitors as Initial Therapy for Interstitial Lung Disease Associated With Antisynthetase Syndrome

This study is a prospective investigation comparing the efficacy and safety of Janus kinase inhibitors versus calcineurin inhibitors as initial therapy for interstitial lung disease associated with antisynthetase syndrome. The goal is to determine which treatment is more effective at improving lung function and preventing disease progression, while comparing their safety profiles. The findings will help provide clearer treatment guidance for doctors and patients.

Efficacy of L-menthol on Breathlessness in Interstitial Lung Disease

The purpose of this study is to assess the effect of L-menthol on breathlessness and exercise capacity in patients with Interstitial lung disease (ILD).

A Study of the Pharmacokinetics and Safety of Single-dose Inhaled RJ026 in Healthy Volunteers and Patients With Interstitial Lung Disease

This Phase 1 clinical trial investigates the pharmacokinetics and safety profile of single-dose inhaled RJ026 in healthy volunteers and patients with interstitial lung disease (ILD). The randomized, double-blind, dose-escalation study employs a parallel-group design with three inhaled dose cohorts (4mg, 8mg, and 12mg) and one oral comparator arm, enrolling a total of 42 patients (12 per inhaled group, 6 in oral group) and 42 healthy volunteers (12 per inhaled group, 6 in oral group). The trial features comprehensive pharmacokinetic sampling through 15 timed blood collections over 24 hours and bronchoalveolar lavage at specified intervals (1h, 6h, 12h, or 24h post-dose) to characterize both systemic and pulmonary drug exposure. The study incorporates rigorous safety monitoring including adverse event tracking, vital sign measurements, and laboratory assessments over a 7-day observation period following drug administration. Conducted at Shanghai Jiao Tong University's Ruijin Hospital over a 12-month period (July 2025-July 2026), this investigation aims to establish the foundational pharmacokinetic parameters and safety profile of RJ026 delivery in ILD patients while comparing pulmonary bioavailability against conventional oral administration.

Acute Hemodynamic Responses to Blood Flow Restriction Aerobic Exercise in Interstitial Lung Disease

Interstitial lung diseases (ILD)impaired gas exchange and reduced lung elasticity lead to marked reductions in exercise capacity and decreased oxygen consumption due to circulatory limitations. Blood flow restriction (BFR) exercise involves applying external pressure to partially restrict venous return without entirely blocking arterial inflow. This controlled compression induces temporary hypoxic and metabolic stress, triggering high-intensity-like responses that stimulate growth hormone release, increase protein synthesis, and promote muscle hypertrophy. However, the most crucial advantage of blood flow restriction during exercise is its ability to increase muscle mass during aerobic training. IIn individuals with ILD, BFR may offer a safe and practical way to improve muscle mass and exercise capacity with minimal additional strain on the cardiovascular and musculoskeletal systems.Our study aimed to compare the acute effects of low-intensity blood flow restriction aerobic exercise training and low-intensity aerobic exercise training on hemodynamic responses and muscle oxygenation in patients with ILD. Method: 30 patients with a diagnosis of ILD being followed up will be included in the study. Our study was a randomized, crossover, triple-blind, prospective study. Assessments will be performed at the beginning of the study. On the first day, demographic data and clinical findings of the individuals will be collected. Patients will be asked questions, and their responses will be recorded in their medical records. Respiratory function, respiratory muscle strength and endurance, and peripheral muscle strength will be evaluated. 48 hours from the first day, patients' maximal exercise capacity will be assessed with a cardiopulmonary exercise test (CPET), and muscle oxygenation during CPET will be assessed with a Moxy® monitor. Respiratory muscle fatigue will be assessed with an oral pressure monitor before and after the exercise test. The assessments will be completed over two days. One week after the evaluations, patients will be randomly assigned to two groups. One group will receive low-intensity aerobic exercise training, and the other will receive low-intensity aerobic exercise training with blood flow restriction. Muscle oxygenation will be assessed during both exercise sessions, and respiratory muscle fatigue will be measured before and after each session. All participants will receive both exercise sessions.

A Study of Mosliciguat in Combination With Inhaled Treprostinil in PH-ILD

This is a Phase 2, open-label, multi-center clinical study to evaluate the safety and efficacy of inhaled mosliciguat in participants with pulmonary hypertension associated with interstitial lung disease (PH-ILD) on a background inhaled treprostinil.

A Study to Test Tetrandrine Tablets for Connective Tissue Disease-Related Lung Disease

This study evaluates the efficacy and safety of tetrandrine tablets (60 mg, three times daily) compared to placebo in adult patients with connective tissue disease-related interstitial lung disease. Patients receive standard treatment (glucocorticoids and immunosuppressants) alongside the study drug or placebo for 24 weeks. The study measures changes in lung function, inflammatory markers, lung imaging, quality of life, and safety outcomes.

Safety Profile of Ultrasound-guided Transparietal Lung Biopsy in Patients With Diffuse Interstitial Lung Disease

Rationale Interstitial lung diseases (ILDs) require accurate histological diagnosis when imaging is inconclusive, yet current techniques-surgical lung biopsy and transbronchial cryobiopsy-carry significant risks and lack real-time guidance. BPTE, widely used in oncology, provides ultrasound-guided sampling under local anesthesia with historically lower complication rates, but its value in ILD remains unproven. The TOUCANS study aims to generate safety and feasibility data to support its potential use as a less invasive diagnostic alternative. Objectives The primary objective is to assess the safety of BPTE in ILD patients, focusing on major adverse events: death, pneumothorax requiring drainage, hemorrhage requiring intervention, and prolonged pleural drainage. Secondary objectives include evaluating all complications within two months, comparing BPTE histological quality with surgical biopsy, and assessing pain, dyspnea, analgesic use, healing, drainage duration, and hematoma on thoracoscopic imaging. Materials and Methods Fifteen patients aged 18-75 with ILD requiring tissue diagnosis after multidisciplinary review will be included. Exclusion criteria cover coagulation disorders, severe comorbidities, long-term oxygen therapy, impaired lung function, obesity, pulmonary hypertension, anesthetic allergy, and vulnerable populations. BPTE will be performed 2-4 weeks before surgical biopsy, under local anesthesia and spontaneous ventilation, with CT- and ultrasound-guided targeting. One or two biopsies will be taken, followed by ultrasound and chest X-ray monitoring; discharge occurs after four hours if stable. Surgical thoracoscopic biopsy will then be carried out with standard postoperative care. Pain, dyspnea, healing, and imaging findings will be recorded, and histological samples will be reviewed blindly. Data will be captured in eCRFs according to GDPR/CNIL requirements. Expected Outcomes TOUCANS will provide the first prospective evidence on BPTE for ILD. Demonstrating safety and adequate diagnostic yield could position BPTE as a minimally invasive, outpatient, ultrasound-guided alternative to surgical biopsy. This may shorten diagnostic pathways, reduce complications, and offer a viable option for patients unsuitable for surgery, ultimately improving early management and prognosis.

Routine vs On-demand ECMO for Lung Transplantation

Lung transplantation is a complex procedure performed in patients with terminal lung disease. The transplant procedure stresses the patient's heart and lungs, which are already taxed by the underlying disease process. The heart-lung machine is occasionally used to support the patient and ensure adequate oxygen supply to other organs during the operation. It can be used routinely in all patients or selectively in patients who exhibit reduced oxygen supply to the remaining organs. This process, known as cardiopulmonary bypass (CPB), pumps blood out of the body to a heart-lung machine that removes carbon dioxide and returns oxygen-filled blood to the body. Although using the CPB increases the risk of bleeding, infection, and coagulation complications, it should still be considered in high-risk patients to compensate for more severe complications such as kidney failure and stroke caused by a lack of cardiopulmonary support. Extracorporeal membrane oxygenation (ECMO) is a recently developed CPB variation associated with fewer bleeding complications. It has recently replaced the traditional heart-lung machine as the preferred method of cardiopulmonary support during lung transplantation. Since ECMO is associated with fewer complications than standard CPB, many centers have increased their use of ECMO during lung transplantation. Some have even employed it routinely. However, there remains significant debate on how often it should be used. Therefore, the study's main objective is to compare the two approaches in lung transplantation, i.e., routine use versus selective use, and to determine if one approach is preferable to the other.

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.

Hypnosis on Breathlessness Mastery in Patients With Persistent Dyspnea

Persistent dyspnea is a debilitating symptom, that is common and difficult to treat. This symptom is found in many different diseases including chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), chronic heart failure (CHF), and metastatic cancer. Dyspnea is associated with many other symptoms, including anxiety, depression, and fatigue and is responsible for a significant reduction in quality of life. To date, only opiates are recommended for the pharmacological treatment of persistent dyspnea. The effectiveness of hypnosis is well known in the treatment of anxiety and delivered to patients suffering from chronic dyspnea for this reason. One randomised controlled study has shown that a single 20-minute mindfulness sessions could significantly reduce general symptom burden and have a significant impact on anxiety and depression in palliative care patients. Furthermore, the intervention had no negative side effects. Our aim is to evaluate the effectiveness of three hypnosis sessions on breathlessness mastery in patients with persistent dyspnea, using as a primary outcome the mastery domain of a validated tool, the Chronic Respiratory Questionnaire (CRQ).

REGEND001 Autologous Basal Layer Stem Cell Transplantation for Interstitial Lung Disease (ILD): A Translational Application Study

This clinical trial evaluates the safety and efficacy of REGEND001, an autologous bronchial basal layer stem cell therapy, in patients with interstitial lung disease (ILD). The treatment involves harvesting the patient's own stem cells (expressing KRT5/P63 markers), expanding them ex vivo, and administering them via bronchoscopic infusion to regenerate damaged lung tissue.

Interstitial Lung Disease Exacerbations Study

Interstitial lung disease (ILD) is an umbrella term covering numerous conditions that affect the lung tissue, interfering with the ability of the lungs to take up oxygen. Most ILDs get worse gradually, but sometimes patients can experience a sudden worsening in their symptoms called an acute exacerbation (AE-ILD). Most studies in this area have been done in AEs of idiopathic pulmonary fibrosis (AE-IPF), as IPF is the commonest form of ILD. AE-IPF has very poor outcomes, however AEs of other ILDs are less well studied. Furthermore, there is currently no treatment guideline or established standard of care for the management of patients with AE-fILD. The aim of this research project is to gain a better understanding of AE-ILD in a real-world population. By looking at the clinical records of patients with AE-ILD, the study aims to describe the patient population that gets AE-ILD and how these patients are treated in the "real world" setting. The study will also gather information on patient characteristics such as type of ILD and test results at the time of AE-ILD, and see if any of these factors are associated with better/ worse outcomes in AE-ILD. Finally, the study will collect data on the treatment approaches taken, including both medical therapy such as steroid treatment, as well as specialist care team input. This data on treatment will be used to identify associations between individual treatments and outcomes, as well as to evaluate the NHS services being provided to patients with AE-ILD. Overall, this study will enhance understanding of AE-ILD. This study will provide information to help design clinical trials to test treatments for AE-ILD, to help us create evidence-based clinical guidelines for AE-ILD, and improve the management of patients with AE-ILD.

Study of the Prevalence of Acid Sphingomyelinase Deficiency/Niemann Pick AB and B Disease in Patients With Diffuse Interstitial Lung Disease

The goal of this clinical trial is to optimise and facilitate screening for Acid SphingoMyelinase Deficiency (ASMD) disease, by evaluating acid sphingomyelinase activity and, where appropriate, LysoSM levels in a cohort of 200 participants with diffuse interstitial lund disease (ILD) at risk of developing ASMD disease. ILD is common in the general population, so in order to limit the number of differential diagnoses, the population to be studied will be restricted to participants aged between 15 years and 3 months and 60 years, with ILD plus ground-glass opacities on chest CT scan certified by a pulmonologist/radiologist or internist, AND splenomegaly or splenectomy, and/or thrombocytopenia, and/or low HDL cholesterol, and/or parental consanguinity which increase the sensitivity of ASMD screening. In this clinical trail, two procedures are added, participants will be asked for : * a blood sample to measure the acid sphingomyelinase enzyme activity and LysoSM, if required. * a follow-up visit at 6 months

Non-Invasive Hemodynamic Monitoring of MV ILD Patients With ARF

Non-invasive hemodynamic monitoring of interstitial lung disease (ILD) patients admitted to respiratory ICU with acute respiratory failure and differentiate according to different modes of mechanical ventilation.

AYLo - AutoimmunitY and Loss of y

The AYLo study (AutoimmunitY and Loss of y - Investigating the Role of Hematopoietic Mutations and Mosaic Mutation in the Y Chromosome in Autoimmune Rheumatologic Diseases) aims to systematically investigate hematopoietic mutations, such as hematopoietic (mosaic) loss of the Y chromosome (mLOY), focusing on their underlying causes, pathophysiological significance, patterns of manifestation, and impact on disease progression in autoimmune, rheumatologic disorders. This research seeks to bridge existing knowledge gaps by exploring how such mutations influence immune homeostasis, cellular function, and susceptibility to inflammation-driven pathologies. Through the integration of advanced immunological profiling, the study aspires to uncover key mechanisms that drive the initiation, progression, and complications of autoimmune rheumatic diseases. These analyses will combine single nucleotide polymorphisms (SNP) arrays, multiplex assays, transcriptomics, and flow cytometry staining of peripheral blood mononuclear cells to delineate the interplay between hematopoietic mutations and immune dysregulation. A further objective is the development of a multimodal framework for disease-specific characterization, enabling precise mapping of mutation-driven phenotypes across diverse autoimmune conditions. This framework will incorporate clinical, molecular, and imaging data. Additionally, the AYLo study aims to explore the potential role of mLOY and other hematopoietic mutations as biomarkers for disease stratification, prognosis, and therapeutic response. The findings may open avenues for personalized treatment approaches, leveraging the molecular insights to inform targeted interventions and improve patient outcomes in autoimmune rheumatic disorders. By integrating translational and basic science approaches, this study has the potential to redefine current paradigms in autoimmune disease research and therapy.

A Trial to Evaluate the Safety, Tolerability and Preliminary Efficacy of NCR101 in the Treatment of Subjects With Interstitial Lung Disease

The target of this trial is to evaluate the safety, tolerability and preliminary efficacy of NCR101 in the treatment of subjects with interstitial lung disease. The trial contains Single ascending dose（SAD） and Multiple ascending dose（MAD）. Subjects will receive at least 1 dose of NCR101.

Fibrotic Interstitial Lung Disease Early Recognition and Strategic Therapy Study in China

This project aimed to: 1) construct a cohort of no less than 10000 cases of f-ILD (including pneumoconiosis ≥3000 cases) with continuous regular follow-up to reveal the clinical phenotypes closely related to the development, progression and prognosis of pulmonary fibrosis; 2) systematically evaluate the safety and effectiveness of frozen lung biopsy, surgical lung biopsy/thoracoscopic lung biopsy and other techniques, and to optimize the histological diagnosis method of f-ILD; 3) construct a set of artificial intelligence (AI) evaluation system for quantitative evaluation of pulmonary fibrosis and its severity, and develop application software; 4) excavate and verify important molecular targets for the formation of pulmonary fibrosis and identify biomarkers; 5) combined with clinical phenotype, imaging, pathology and biomarkers to establish f-ILD early recognition and progress model, intervention strategies, guidelines and consensus, and applicated nationwide.