Ingo Ganzleben, Benjamin D. Medoff
While certain pathologic changes in the ECM, such as fibrotic foci, have long been recognized as hallmarks of pulmonary fibrosis, the matrix has only recently been recognized as an important factor in precipitating fibrosis inception and progression rather than just being an endpoint of fibrotic disease.
The extracellular matrix (ECM) is a crucial component of the lung, providing both tissue stability and elasticity. While certain pathologic changes in the ECM, such as fibrotic foci, have long been recognized as hallmarks of pulmonary fibrosis, the matrix has only recently been recognized as an important factor in precipitating fibrosis inception and progression rather than just being an endpoint of fibrotic disease. In this review, we summarize the concept of the ECM as an active player in fibrogenesis, new insights into its qualitative and quantitative composition in pulmonary fibrosis, as well as how cells sense physical matrix properties. Beyond that, we give an overview of cutting-edge technical approaches to study matrix and cell mechano-biology, and, lastly, we summarize current advances in developing mechano-therapeutics to treat fibrotic diseases.