Extracellular matrix (ECM) plays a crucial role in the regulation of both physiological and pathological angiogenesis. ECM homeostasis and function is ensuring by the tightly regulation of the different ECM components including, collagens, proteoglycans and a variety of different glycoproteins. An altered expression of the above ECM molecules as well as an imbalance between the action of matrix remodeling enzymes and their tissue inhibitors is known to be responsible for impaired angiogenesis and fibrosis. Systemic Sclerosis (SSc) is an autoimmune disease characterized by micro-angiopathy, failure of reparative angiogenesis, and excessive fibrosis of the skin and various internal organs, dues to an increased production of ECM. A comprehensive search through Medline/PubMed and Scopus was performed for English-language original papers, using the keywords related to ECM components and SSc. This review will analyze the role played by ECM components in the deregulation of angiogenic mechanisms and in the persistence of a pro-fibrotic phenotype, during SSc. A better knowledge of these processes might provide information about molecules, which could be considered targets for future pro-angiogenic and/or anti-fibrotic therapies.
Keywords: Angiogenesis; extracellular matrix; fibrosis; systemic sclerosis; vasculopathy.