Active clinical trials for "Idiopathic Pulmonary Fibrosis"

While the bidirectional relationship between the lung and the right heart are well studied, the cardiopulmonary interactions between the lung and the left heart are largely unresearched and not well understood. However, in recent years, there is a growing evidence that partially explains the bidirectional interaction between COPD and left heart. Systemic inflammation with multiorgan involvement is thought to play a role in COPD as a systemic disease. Some therapeutic approaches to COPD also appear to influence these cardiopulmonary interactions. While understanding these interactions is very important for clinicians, scientific data are scarce. Cardiac magnetic resonance imaging (cardiac MRI) is the gold standard for assessing cardiac function and dimensions as well as myocardial inflammation. Despite this excellent suitability of cardiac MRI for the assessment of cardiovascular function, only few studies have investigated cardiac function and myocardial structure in patients with pulmonary disease using cardiac MRI. Such a study is therefore very important for understanding the effects of pulmonary disease and its management on the heart. The objective is to determine cardiac function in patients with pulmonary disease and to analyze the cardiovascular effects of the treatment of the pulmonary disease. Specifically, the following will be studied: Using cardiac MRI: Cardiac function and volumes and indications of myocardial fibrosis and edema in patients with chronic pulmonary disease at the time of first diagnosis. the vascular function of pulmonary arteries in these patients, also using cardiac MRI the relationship between pulmonary function parameters and cardiac dysfunction to identify patients at increased risk, if applicable. Echocardiographic assessment of left heart including strain analysis. the course of these cardiovascular parameters (using cardiac MRI and echocardiography) 3-6 months after initiation of guideline-based therapy for pulmonary disease.

Idiopathic pulmonary fibrosis (IPF) is characterized by a poor prognosis, with a progressive decline in lung function and a considerable variability in the disease's natural history. Besides lung transplantation (LTx), the only available treatments are anti-fibrosing drugs, which have shown to slower the disease course. Therefore, predicting the prognosis is of pivotal importance to avoid treatment delays, which may be fatal for patients with a high risk of progression. Previous studies showed that a multi-dimensional approach is practical and effective to create a reliable prognostic score for IPF. In the RIsk Stratification scorE (RISE), physiological parameters, an objective measure of patient-reported dyspnea and exercise capacity are combined to capture different domains of the complex pathophysiology of IPF. This is an observational, multi-centre, prospective cohort study. A development cohort and a validation cohort will be included. Patients newly diagnosed with IPF based on the ATS/ERS criteria and multi-disciplinary discussion will be included in the study. A panel of chest radiologists and lung pathologists will further assess eligibility. At the first visit (time of diagnosis), and every 4-months, MRCDS, pulmonary function tests (FEV1, FVC and DLCO), and 6MWD will be recorded and patients will be prospectively followed for 3 years. Comorbidities will be considered. The radiographic extent of fibrosis on HRCT will be recalculated at a 2-year interval. RISE, Gender-Age-Physiology, CPI and Mortality Risk Scoring System will be calculated at 4-month intervals. Longitudinal changes of each variable considered will be assessed. The primary endpoint is 3-year LTx-free survival from the time of diagnosis. Secondary endpoints include several, clinically-relevant information to ensure reproducibility of results across a wide range of disease severity and in concomitance of associated pulmonary hypertension, emphysema. The present study aims at validating RISE as a simple, straightforward, inexpensive and reproducible tool to guide clinical decision making in IPF and potentially as an endpoint for future clinical trials.

This is a multicentric, randomised, double-blind, placebo-controlled study that will consist of two consecutive phases: First phase: faecal samples will be collected in patients diagnosed with Idiopathic pulmonary fibrosis treated with nintedanib. Second phase: double-blind, randomised, clinical trial of autologous faecal microbiota transplantation (FMT) vs placebo in Idiopathic pulmonary fibrosis patients who will experience nintedanib-induced diarrhea within 8 weeks of baseline visit. Follow-up visits will be scheduled at 1, 4 and 12 weeks after randomization. The main aim of the study is to assess the efficacy of FMT in ameliorating diarrhea experienced by patients with idiopathic pulmonary fibrosis treated with nintedanib.

This is a multi-center randomized, double-blind, placebo-controlled, parallel, 4-arm study of nalbuphine ER (NAL ER). After meeting eligibility during the Screening Period, subjects will be randomized (1:1:1:1) to one of four treatment arms. Arm 1: Placebo Arm 2: 27 mg nalbuphine ER Arm 3: 54 mg nalbuphine ER Arm 4: 108 mg nalbuphine ER Each arm will be titrated to their fixed dose during the blinded 2-week Titration period followed by the 4-week Fixed Dose Period for a total of 6 weeks on drug.

The goal of this clinical trial is to learn about INS018_055 in adults with Idiopathic Pulmonary Fibrosis (IPF). The primary objective is to evaluate the safety and tolerability of INS018_055 orally administered for up to 12 weeks in adult subjects with IPF compared to placebo.

The study will assess the role of using azithromycin in managing acute exacerbation of Idiopathic pulmonary fibrosis

This study is a randomized, double-blind, placebo-controlled, multi-center phase II clinical study conducted in China to compare the efficacy and safety of two different dose groups of AK3280 in IPF patients compared to the placebo control group.

In this study the prognostic value of the current screening parameters for familial pulmonary fibrosis (FPF) will be investigated by looking at the screenings of 200 first-degree relatives of patients with FPF. Also insight in the natural history of early FPF, and the necessary interval between screenings visits will be investigated.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with a poor prognosis. More accurate tests to predict disease progression and response to treatment are required. Krebs von den Lungen-6 (KL-6) is a blood marker associated with IPF. Results from previous studies have shown that levels of KL-6 are higher in patients with IPF compared to people without the disease. In addition, it is not clear what impact treatment has on KL-6 levels, and whether this could help us to monitor how effective treatment for IPF is. The investigators plan to perform a study in which KL-6 levels in the blood of patients with a new diagnosis of IPF are measured at baseline, 3, 6 and 12 months to look for and changes in the levels of KL-6 in the blood.

A complex interaction between demographic, environmental and genetic mechanisms impact the onset, severity and outcome of ILD-SARDs through dysregulation of the immune system and lung pro-biotic pathways. Comorbidity and genetic risk indicate that there are overlapping pathogenic mechanisms among SARDs, some of which underlie ILD in different SARDs. The purpose of this biobank is to study the clinical, pathological, laboratory, and imaging characteristics of SARDs patients with lung involvement. This will help identify as unique features underlying lung involvement in SARDs. In addition, this may lead to the discovery of novel mechanisms of disease and potentially novel targets of treatment for SARDs patients with lung disease.