Researchers from Helmholtz Munich in cooperation with an international team have discovered a key mechanism that contributes to the development of idiopathic pulmonary fibrosis. This could be a target for future diagnostic and therapeutic methods for this serious and usually chronic disease.
Idiopathic pulmonary fibrosis (IPF) is a rare and progressive disease in which the lung tissue becomes scarred. Functional lung tissue is increasingly remodelled into non-functional connective tissue. This scarring impairs the ability of the lungs to transport oxygen into the bloodstream, resulting in breathlessness and a reduced oxygen supply to the body. Healthy lungs can repair damage and regenerate. This ability is impaired in lung diseases such as pulmonary fibrosis. To date, two active substances have been approved for the treatment of pulmonary fibrosis.
Mechanism discovered that inhibits lung regeneration
Researchers from Helmholtz Munich, together with an international team, have now discovered a process that reduces the regenerative capacity of lung stem cells in pulmonary fibrosis. Earlier studies by the same research team had already shown this: If more so-called extracellular vesicles are released, this promotes the progression of pulmonary fibrosis.
Extracellular vesicles are small, cell-derived particles that facilitate communication between cells. They are released by connective tissue cells in the lungs, known as pulmonary fibroblasts. These specialized cells drive the scarring process in pulmonary fibrosis.
The scientists have now discovered that extracellular vesicles containing the protein Secreted Frizzled-Related Protein 1 (SFRP1) accumulate during active fibrosis. This impairs the regenerative capacity of the lung stem cells.
Possible biomarkers for early detection
The SFRP1-containing extracellular vesicles can be detected in bronchoalveolar lavage fluid. This can be obtained relatively easily as part of a lung endoscopy. The small particles could therefore serve as biomarkers for the early detection of pulmonary fibrosis.
In further studies, the research team now wants to investigate whether this mechanism can also be used to restore the lung's ability to regenerate.