[Pathophysiologic aspects of systemic sclerosis].
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.
Gran
Jan Tore
JT
Revmatologisk avdeling, Rikshospitalet 0027 Oslo. jan.tore.gran@rikshospitalet.no
Molberg
Øyvind
Ø
Midtvedt
Øyvind
Ø
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
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