Lung Fibrosis Interstitial

The purpose of this interventional study is to identify which combination of remote monitoring devices (e.g. home spirometry, pulse oximetry, scale, ePROs) is the most feasible (as defined by adherence, retention, and data completeness) and acceptable when used for the detection of clinically significant Interstitial Lung Disease events.

The goal of this observational study is to evaluate the feasibility and acceptability of a newly developed digital education and self-management program for people with fibrotic interstitial lung diseases (FILD) during inpatient pulmonary rehabilitation and outpatient interstitial lung disease (ILD) care. The study also explores whether participation in the program is associated with changes in health-related outcomes. The main questions this study aims to answer are: * Is the digital education and self-management program acceptable, useful, and practical for patients with FILD? * Is it feasible to recruit and retain patients and to collect outcome data for a future larger effectiveness study? * Do patients who participate in the program show differences in health-related outcomes compared with patients treated before the program was implemented? Patients who consent to participate during the implementation phase will be offered access to the digital education and self-management program. Their outcomes will be compared with those of a historical cohort of patients who received usual care before the program was introduced. Participants will: * Use a web-based digital education and self-management program with multiple e-learning modules tailored to individual needs and preferences * Access the program via smartphone, tablet, or computer during inpatient rehabilitation or outpatient care * Discuss questions related to the program content with healthcare professionals during routine rehabilitation sessions or clinic visits * Complete questionnaires about their experience with the program and their health-related outcomes

The PROMPTLY study is a proof-of-concept research project evaluating the efficacy of XHALIP, an innovative inhalable formulation of Imatinib, in treating pulmonary arterial hypertension (PAH) and post-inflammatory fibrosing lung diseases (LFD). Main Objective To demonstrate the ability of XHALIP to improve the prognosis of PAH and LFD by reducing pulmonary fibrosis and vascular remodeling. Key Research Questions Is XHALIP effectively absorbed by pathological lung cells? Does it have a beneficial biological effect on cell proliferation and extracellular matrix deposition? Study Plan Preclinical phase: In vitro tests on cells obtained from patients with PAH and LFD. Absorption and distribution analysis: In vitro lung tissue models to assess drug penetration and effectiveness. Biological activity assessment: Evaluating XHALIP's ability to inhibit epithelial-mesenchymal transition and cell proliferation. Participants Patients aged ≥ 18 years diagnosed with PAH or LFD, either candidates for or recipients of lung transplantation. Biological samples (bronchoalveolar lavage and explanted lung tissues). Methodology Confocal microscopy and flow cytometry to analyze absorption. RT-PCR and Western Blot to assess biological efficacy. Alveolo-capillary models to test drug release and distribution. The study aims to translate preclinical findings into a potential future clinical trial for the development of XHALIP as a novel inhalation-based therapeutic strategy for rare lung diseases.