Hyaluronic Acid is Overexpressed in Fibrotic Lung Tissue and Promotes Collagen Expression

摘要

14. ABSTRACT In 30 to 70 % of systemic sclerosis patients, the disease progresses to the lungs and internal organs resulting in tissue damage and remodeling, inflammation, and fibrosis (i.e. the overexpression of collagen). Lung fibrosis is the major cause of morbidity and mortality in scleroderma and is particularly devastating because there are no FDA-approved treatments. The overexpression of collagen is accompanied by the overexpression of other extracellular matrix molecules including periostin. Periostin is of particular interest because it regulates inflammatory cell infiltration and the differentiation of fibroblasts (the cell type that expresses collagen in fibrotic tissue) in other systems. For these reasons, we are determining whether periostin plays a regulatory role in the progression of lung injury/fibrosis, possibly by regulating inflammation or possibly by regulating the differentiation of fibroblasts and their expression of collagen. Indeed, our recent experiments using periostin knockout mice validate the importance of this molecule in that lung injury/fibrosis in these mice is worse than in control mice. The results of these studies may suggest novel therapies for lung fibrosis in which the function(s) of periostin are altered.