Different Cytokines and Growth Factors Have Different Expression in Limited or Diffuse Systemic Sclerosis.
Edwin R Parra, Leticia O Silva, Maristela Rangel, Sandra M Fernezlian, Vera L Capelozzi. Faculdade de Medicina da Universidade de São Paulo, Brazil
Background: Systemic sclerosis (SSc) is a multisystem connective tissue disorder characterized by excessive accumulation of extracellular matrix, resulting in progressive fibrosis and dysfunction of a number of organs. In this regards many studies suggest that several cytokines and growth factors have been implicated in the SSc. The aim of this study was examined different factors, cytokines and growth factors, implicated in the fibrotic process in limited and diffuse SSc.
Design: Lung specimens were obtained from 23 patients with limited (n=10) or diffuse (n=13) SSc. Immunohistochemical staining of alpha-smooth muscle actin (α-SMA), anti-interleukin (IL)-4, anti-IL-13, anti-basic fibroblast growth factor (bFGF), anti-transforming grow factor beta (TGF-β), anti-telomerase and anti-endothelin-1 (ET-1), and morphometric analysis were used to score in epithelial, endothelial, myofibroblast and smooth muscle cells and correlated with pulmonary functions.
Results: We observed higher epithelial and bronchiolar smooth muscle cells expression of bFGF and higher endothelial IL-4 and IL-13 expression in diffuse SSc when compared with limited SSc (p<0.05). A significant negative association existed between myofibroblast ET-1 expression and VC (r=-0.594, p=0.02); endothelial bFGF expression and DCO (r=-0.583, p=0.03); and a positive association between endothelial telomerase expression and FEV1, FVC, and DCO/VA (r=0.435, p=0.04; r=0.585, p=0.02; and r=0.595, p=0.02; respectively).
Conclusions: The evidence suggests that increased from epithelial and bronchiolar smooth muscle cells expression of bFGF and endothelial IL-4 and IL-13 expression in diffuse SSc can be partially mediated by the extension of the disease, but more importantly provide a potential histochemical biomarker for differentiating limited SSc from diffuse SSc. However, further studies are needed to determine whether or not these overexpressions are causative or indicative of SSc progression.
Financial support: FAPESP, CNPq.
Wednesday, March 2, 2011 9:30 AM
Poster Session V # 261, Wednesday Morning