Active clinical trials for "Lung Diseases, Interstitial"

The purpose of this study is to establish a clinical database for patients bearing at risk for ILD (Interstitial Lung Disease) and to set up a prospective ILD Screening program for these patients.

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.

We propose to acquire data and blood samples on all patients being cared for by the Interstitial Lung Disease (ILD) program. Additionally, we will collect data and blood samples from a control group for comparator purposes. In doing so, we will be able to describe the "phenotypic" expression of these diseases.

Despite a number of prospective studies already initiated in the past years, the current epidemiology and course of interstitial lung disease (ILD) and pulmonary hypertension (PH) in patients with connective tissue disease (CTD) is still not well defined, particularly regarding its prevalence, incidence and the management of a broad spectrum of disease presentations. Major challenges include the identification of patients with progressive disease, the appropriate time point of therapeutic intervention and the underlying driver of disease (inflammatory or pro-fibrotic stimulus or both?). To address these issues in Western Austria, a progressive registry of patients with CTD exploring routine clinical and pathophysiological characteristics of ILD and PH will be conducted. This multidisciplinary, prospective and observational registry aims to collect comprehensive clinical data on incidence, prevalence and course of disease regarding all PH and ILD presentations in a real-world setting.

Integrating Molecular, Genomic, Morphology and Environmental Features to Improve Precision Diagnosis and Treatment in Interstitial Lung Diseases (PRECISION-ILD) Background: Interstitial Lung Diseases (ILDs) are a heterogeneous group of >100 different, rare diseases, which share the fate of progressive scarring and, ultimately, death. Two anti-fibrotic drugs have demonstrated to slow-down fibrotic progression and steroids/immunosuppressants are commonly used for inflammatory-driven ILDs. However, patient's response to therapeutic options is variable and unpredictable. Similarly, setting a correct diagnosis is difficult in most cases, especially when patients are too sick for invasive procedures. Objectives: (1) To investigate the differences and commonalities in genetic, genomic and environmental exposures/lifestyle in fibrotic ILDs depending on the entity, disease behavior (progressive fibrosis) and treatment response; (2) To integrate the biomarkers that most impact on prognosis and treatment response in diagnostic algorithms; and (3) To explore the feasibility and cost of implementing a P4 strategy in clinical practice for fibrotic ILDs. Methods: The investigators will extend, update and unify existing ILD cohorts (Spanish SEPAR ILD Reg, Observatory IPF.cat, CIBERES IPF and Familial ILD cohorts) in whom the researchers will: (1) record demographic, epidemiological, clinical, physiological and lung morphology (radiological +/- histological) information; (2) obtain genetic variation, telomere length, and serum protein markers; (3) investigate environmental exposures (including air-pollution), (4) apply to integrative analytical methods to identify endotypes, predictive biomarkers of disease trajectories, theragnostic biomarkers and new therapeutic targets. Results (5) will be validated in other fibrotic ILD cohorts (e.g.EuILDRegistry, Mexican fibrotic ILD Registry). Besides, the investigators will explore how to translate this P4 medicine approach in clinical practice; (6) implementing a predictive score for prognosis and improving the diagnostic approach through biological data to reduce invasive procedures, and (7) estimate educational requirement and potential health cost implications. Viability:This project is viable because: (1) cohorts already exist and can be expanded and updated; (2) investigators have ample expertise in translational research and actively participate in ILD consortia; (3) required knowledge and methodology is already in being used by the consortium. Clinical relevance: Due to the lethality, high social and economic burden of fibrotic ILDs, identifying the best diagnostic and therapeutic approach through preventive, personalized and precise measures is a unique opportunity to improve survival in these patients and efficiency of health-care resources.

The aim of this study is to assess the findings of mediastinal/hilar LN sampling by EBUS-TBNA in patients with non-sarcoidosis interstitial lung disease ( ILD) who demonstrate LN enlargement on chest imaging. Patients with non-sarcoidosis ILD referred for bronchoscopy will undergo LN sampling by EBUS-TBNA. Cytology results will be recorded along with clinico-radiologic features, BAL findings, histology and final ILD diagnosis.

Rheumatoid arthritis (RA) is one of the most common autoimmune diseases, characterized by chronic inflammatory bone and cartilage destruction. Although treatment including anti-tumor necrosis factor (TNF) and interleukin-6 (IL-6) receptor antibodies has been successful, only 20% to 30% of patients have achieved complete remission. Interstitial lung disease (ILD) is a common complication of rheumatoid arthritis (RA). Approximately 5-10% of RA patients have clinically significant rheumatoid arthritis associated interstitial lung disease (RA-ILD), with a mortality rate of 2-10 times that of RA-non ILD patients. The median survival after diagnosis is between 3-8 years. Although there are multiple biomarkers for RA-ILD, such as anti citrullinated protein antibody (ACPA), MUC5B mutant gene, KL-6, etc., none of these biomarkers can reliably predict the disease and prognostic risk of RA-ILD. Therefore, improving the prediction of RA complicated with ILD and exploring risk factors for the progression and prognosis of RA-ILD can contribute to early diagnosis and treatment, and is of great significance in preventing RA lung injury and death. This study aims to screen differential serum biomarkers between RA patients and RA-ILD patients through prospective cohort studies, to explore whether these differential serum biomarkers are a risk factor for RA patients complicated with ILD, and whether they affect the clinical prognosis of RA-ILD patients.

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 purpose of this study is to investigate new therapies for advanced lung disease.

Participants with respiratory disease experience often a worsening of their condition, with increasing symptoms such as cough and shortness of breath. This worsening, often called exacerbation or flare up, impacts on the life of the participants, since they become limited in their daily activities. Healthcare is still based today on limited times for clinical appointments to perform investigations and to meet with specialists/clinicians. Very often, these evaluations do not reflect the way the disease is limiting the patient's life. Wearable devices offer the opportunity to collect data on physical activities and important clinical parameters (such as how the patient is active or just staying in bed during the day), on a daily basis. The HG Phoenix AI- based Smart Watch produced by Health Gauge, an Albertan company based in Edmonton, has the potential to measure heart rate, heart rate variability, blood pressure, pulse wave velocity, respiratory rate, temperature, arterial saturation, sleep pattern (deep, light sleep, awake time), duration and time, daily physical activities (site count and distance) and calories burnt in a simple and non-invasive fashion. Ideally, these parameters could be monitored and recorded 24 hours per 7 days per week. This study aims to demonstrate that this device can be used for a long time at home and it is comfortable to use for the participants, that it is not dangerous and, possibly, that it can help to identify exacerbations before the currently available investigations.