Overview
Chronic lung diseases such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD) can lead to pulmonary hypertension. This serious complication involves increased pressure in the lung vessels, which strains the heart and worsens outcomes. Since the early symptoms are unclear, diagnosis often occurs too late, underscoring the need for simple, noninvasive methods of early detection.
A key driver of the disease is vascular remodeling, which involves the narrowing and stiffening of blood vessels. This process involves changes in the extracellular matrix, particularly in the understudied basement membrane. Our project examines how specific components, especially non-classical collagens, change during disease progression. As vessels remodel, detectable fragments enter the bloodstream, potentially creating a molecular fingerprint of the disease.
By analyzing lung tissue and blood samples, the investigators aim to identify non-invasive biomarkers for earlier diagnosis, better patient classification, and more personalized treatment.
Description
Pulmonary hypertension (PH) is a severe complication of chronic lung diseases (CLDs) that significantly worsens patient outcomes. Although extracellular matrix (ECM) remodeling is central to PH pathogenesis, the basement membrane (BM)-a specialized ECM-remains understudied. The BM's dynamic role in lung tissue organization and signaling may provide insight into disease progression and phenotypic heterogeneity.
The investigators hypothesize that BM remodeling and the release of its bioactive components generate a measurable molecular fingerprint reflecting PH onset and progression in CLD. This study aims to prove the concept of using BM-derived fingerprints for disease detection and patient stratification.
Eligibility
Inclusion criteria:
Patients undergoing lung transplantation with COPD or PF, with or without associated PH and PAH.
\- Outpatient cohort with COPD or PF, with or without associated PH or PAH.
Exclusion criteria:
- Presence of other lung diseases
- Signs of infection, such as pneumonia, pulmonary tuberculosis, or pleural effusions.
