Interstitial Lung Disease Ild

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.

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.

Pulmonary rehabilitation is a key treatment for lung diseases, but many patients struggle to attend sessions due to travel distances, physical limitations, or logistical barriers. The purpose of this study is to evaluate whether a tele-pulmonary rehabilitation (TPR) program can improve the rates of treatment initiation and adherence among patients with chronic lung diseases compared to traditional, center-based pulmonary rehabilitation. Additionally, the study will assess improvements in quality of life, physical symptoms, and safety in both groups. This study will enroll 90 patients from two medical centers in Israel (Tel Aviv and Barzilai). Participants will be randomly assigned to one of two groups: 1. The control group will receive usual care with a referral by a pulmonologist to standard pulmonary rehabilitation at a medical center. 2. The intervention group will participate in a supervised remote tele-rehabilitation program using a dedicated application and remote monitoring.

Breathlessness is a complex symptom that results in poor quality of life, increased hospitalisations and increased mortality. Breathlessness is influenced by physiological, psychological and functional factors but these are poorly explored. There are also unexplored phenomenon's such as breathing pattern. Therefore, this study aims to understand the influences of physiology, psychology, function and breathing pattern on health related quality of life in those with a respiratory disease compared to healthy controls. This project is a cross-sectional cohort study including those with a known respiratory disease and experiencing breathlessness, compared to non-breathless healthy controls. The investigators will measure your physiology through spirometry (a breathing test that tells us about their lung function), psychology through questionnaires, function through an exercise test and breathing pattern using opto-electrictronic plethysmography (markers are placed on your chest to see how participants breathe while exercising). This will be conducted over two visits. The investigators will recruit participants from clinics at the University Hospitals of Leicester NHS Trust. The investigators are aiming to recruit 50 participants with a respiratory disease and 25 healthy controls. The results of this study will help us understand breathlessness in more detail in order to be able to develop better treatments.

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).

This is a prospective, multicenter, observational cohort study enrolling approximately 4,000 subjects across about 50 centers, including patients with COPD (confirmed and suspected), asthma (confirmed and suspected), bronchiectasis, interstitial lung disease (ILD), upper airway obstruction (UAO), and healthy controls. Participants will undergo standardized clinical assessments, cough search, FENO, ETCO2, impulse oscillometry, spirometry, and/or bronchodilator test, and/or bronchoprovocation test, and/or diffusion capacity test at a single baseline visit, with no investigational intervention or treatment assignment. The primary objective is to evaluate the diagnostic performance of portable impulse oscillometry in Chronic respiratory diseases and to develop an artificial intelligence diagnostic model for COPD based on oscillometry. The study duration per subject is limited to the screening/baseline visit, with no follow-up visits planned.

This project aims to create and improve a VR-based breathing exercise program for patients with interstitial lung diseases (ILDs). It involves three phases: (1) developing an interactive VR application with guided breathing exercises and real-time biofeedback tailored for ILD patients, (2) testing its feasibility by evaluating usability and acceptability, and (3) refining the intervention based on feedback from the feasibility study.

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.

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.

Patients with interstitial lung diseases (ILD) suffer significant delays in obtaining a diagnosis, and objective evidence of support for immunosuppressive therapies, due to weaknesses in the diagnostic tests available in ILD clinics. This project will assess several non-invasive assessments, focusing on blood and breath based sampling, to identify candidate tests that may translate through to routine clinical use. The tests being assessed will be initially employed in Pigeon Fanciers, a group recognised to be at high risk of developing ILD compared to age matched controls, due to their regular exposure to a known cause of ILD. Candidate tests will also be assessed for change over time in this group. Tests which show promise will then be utilised in patients with ILD diagnoses, including those with severe respiratory failure, to assess their acceptability to patients and their ability to provide useful prognostic information, to allow powering of future definitive clinical trials.

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.

This is a trial designed to evaluate the combination of nerandomilast with mycophenolate across a wide variety of pulmonary fibrosis subtypes, with the aim of providing clinicians with assurance that this is an appropriate therapeutic combination.

This is an exploratory, randomized, single-blind, placebo-controlled, multiple-dose, dose-escalation clinical study. The study aims to evaluate the safety and preliminary efficacy of nebulized inhalation of Human Umbilical Cord Mesenchymal Stem Cell-Derived Extracellular Vesicles (hUCMSC-EVs) in patients with interstitial lung disease (ILD). This study is designed to include three sequential dose cohorts-low, medium, and high-with dose escalation proceeding in an ascending order. Eligible subjects will be enrolled and randomized sequentially, with a planned enrollment of 8 subjects per cohort. Within each cohort, subjects will be randomly assigned in a 3:1 ratio to receive either hUCMSC-EVs or saline placebo.

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).

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). The long-term benefits of using ICBs as a second-line therapy are now clear. 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.

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

This clinical trial is intended for patients awaiting an interventional bronchoscopy procedure where the use of a cryocatheter is considered. This type of catheter is already used in clinical practice for the treatment of certain types of bronchial obstructions, including tumors, granulomas, and for foreign bodies extractions. This catheter uses extremely cold temperatures offering unique advantages compared to heat-based technologies. Different types of reusable and single-use cryocatheters are actually available on the market. This type of device requires the use of expensive and bulky consoles operating with heavy gas tanks, resulting in high maintenance costs. A Quebec, Canada based company has developed a cryocatheter that combines procedural efficacy with practical considerations in mind. The Celsio cryocatheter operates without a console and is designed to accommodate the bronchscopist through the different types of cryogenic procedures. This study aims to generate meaningful, real-world evidence without interfering with standard clinical care. The collected data will serve to characterize the device's performance profile, identify best practices for its use, and provide data for the design of future prospective comparative studies.

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.

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.

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.

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.

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.

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.

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.

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.