Study of pulmonary vasculopathy in experimental model of systemic sclerosis induced by type V collagen

• Grant number: 10/17779-2
• Support Opportunities: Regular Research Grants
• Start date: January 01, 2011
• End date: December 31, 2012
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal Investigator: Walcy Paganelli Rosolia Teodoro
• Grantee: Walcy Paganelli Rosolia Teodoro
• Host Institution: Faculdade de Medicina (FM). Universidade de São Paulo (USP). São Paulo , SP, Brazil
• Associated researchers: Ana Paula Pereira Velosa ; Claudia Goldenstein Schainberg ; Francisco Garcia Soriano ; Hermes Vieira Barbeiro ; Natalino Hajime Yoshinari ; Roberta Gonçalves Marangoni ; Vera Luiza Capelozzi

Abstract

Systemic sclerosis (SSc) is autoimmune disease with unknown cause, characterized by autoimmunity, vasculopathy and fibrosis with tissue remodeling, progressing to tissue destruction and organ failure. Pulmonary involvement, characterized by lung interstitial fibrosis and pulmonary hypertension, is the leading cause of death. The initial cellular alteration of SSc lung disease is characterized by inflammatory septal hyperplasia, myofibroblasts proliferation, capillaries revascularization in the microcirculation, and increased endothelial activity evidenced by high expression of adhesion molecules. The endothelial lesion is described to be the initial pathogenic event and results in increased vascular permeability, attraction of inflammatory cells and production of cytokines and vasoactive mediators. The perpetuation of the initial immunologic stimuli promotes extracellular matrix overproduction by fibroblasts activation, resulting in collagen accumulation. At the present moment, the underlying pathogenesis of SSc is still incompletely understood. Our purpose is to evaluate the role of endothelium in the animal model of systemic sclerosis, aiming to understand the different stages involved linking endothelial activation and matrix remodeling. (AU)