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SP0177 Linking Vasculopathy and Fibrosis
  1. M. Trojanowska
  1. Arthritis Center, Boston University, Boston, United States

Abstract

The hallmark features of SSc include fibroproliferative vasculopathy, immune dysfunction, and extensive skin and organ fibrosis. Delineation of the common pathways responsible for the pathological changes affecting the key disease cell types would be critical to developing effective therapeutic strategies. Extensive clinical and experimental evidence implicate alterations of transcription factor Fli1 (Friend leukemia integration-1) in disease pathogenesis. Fli1 deficiency contributes to the induction of SSc-like phenotypic changes in dermal fibroblasts, endothelial cells and macrophages in a manner consistent with human disease. Thus, restoring the Fli1 levels in diseased tissues may represent a valid therapeutic strategy to treat SSc. Elucidation of factors that contribute to Fli1 deficiency in SSc will be essential to fully implement this strategy. This presentation will discuss the progress in characterizing such factors and potential therapeutic strategies to restore Fli1 levels.

Disclosure of Interest None declared

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