title: [Pathophysiologic aspects of systemic sclerosis].
abstract: BACKGROUND
  The pathological changes in systemic sclerosis are characterised by severe organ fibrosis, obliteration of arteries and arterioles and disturbance of immunological functions. Recent research has yielded new insight into the complex pathogenesis of systemic sclerosis, with new therapeutic options as a possible consequence.
  
  
  METHODS
  Relevant medical literature has been reviewed.
  
  
  RESULTS
  The pathological deposition of extracellular matrix in systemic sclerosis is most probably caused by changes in the regulation of dermal fibroblasts. The review focuses on three of the many molecules involved in the regulation of fibrosis; Transforming growth factor beta, Connective tissue growth factor and Endothelin-1.
  
  
  CONCLUSIONS
  Treatment of organ-specific disease complication has for long remained the only therapeutic option in systemic sclerosis. Based on current knowledge of the process of fibrosis, new therapeutic trials employing substances directed towards pro-fibrotic molecules are now in progress.
authors.affiliation: Revmatologisk avdeling, Rikshospitalet 0027 Oslo. jan.tore.gran@rikshospitalet.no
authors.forename: Jan Tore
authors.initials: JT
authors.lastname: Gran
authors.forename: Øyvind
authors.initials: Ø
authors.lastname: Molberg
authors.forename: Øyvind
authors.initials: Ø
authors.lastname: Midtvedt
mesh_terms: D055513:Connective Tissue Growth Factor; D019332:Endothelin-1 / Q000378:metabolism; D016232:Endothelins / Q000378:metabolism; D005347:Fibroblasts / Q000378:metabolism; D006133:Growth Substances / Q000378:metabolism; D006801:Humans; D017874:Immediate-Early Proteins / Q000378:metabolism; D036341:Intercellular Signaling Peptides and Proteins / Q000378:metabolism; D012595:Scleroderma, Systemic / Q000150:complications / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D016212:Transforming Growth Factor beta / Q000378:metabolism
chemical_list: C526317:CCN2 protein, human; D019332:Endothelin-1; D016232:Endothelins; D006133:Growth Substances; D017874:Immediate-Early Proteins; D036341:Intercellular Signaling Peptides and Proteins; D016212:Transforming Growth Factor beta; D055513:Connective Tissue Growth Factor