Rheumaderm pp 93-100 | Cite as

Raynaud’s Phenomenon and Vascular Disease in Systemic Sclerosis

  • S. Generini
  • M. Matucci Cerinic
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 455)


Raynaud’s phenomenon (RP) is very often the first manifestation of SSc preceding the onset of all the other signs and symptoms of the disease. Two structures are involved in the pathogenesis of RP: the endothelium and the peripheral nervous system (PNS). The hypothesis is that SSc modifies consistently the activity of both these systems leading eventually to RP. The disease, through the injury to the endothelium, jeopardizes the basilar endothelial-dependent vascular tone control. An increase of endothelin, a potent endo- thelial-derived vasoconstrictor, and the reduction of nitric oxide, one of the main endothelial vasodilators, are two key events involved in the genesis of RP. The PNS is also targeted by the disease as demonstrated by the high incidence of neuropathy in SSc patients. A marked reduction of sensory fibres has been detected in SSc skin. Thus, the involvement of nerve terminals reduces the vasodilatory, endothelial dependent or independent, potential of the neuropeptides released by sensory nerve endings. Indeed, an increased sensitivity of α2 adrenoceptors mediated vasoconstriction has been shown in SSc skin. The complex vasodilatory network formed by the interaction between the endothelium and the PNS seems greatly damaged by SSc leading inesorably toward vascular tone dysfunction clinically evident as RP.


Systemic Sclerosis Vasoactive Intestinal Peptide Calcitonin Gene Related Peptide Sensory Nerve Ending Angiotensin Converting Enzyme Plasma Activity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media New York 1999

Authors and Affiliations

  • S. Generini
    • 1
  • M. Matucci Cerinic
    • 1
  1. 1.Department of Medicine Division of RheumatologyUniversity of FlorenceItaly

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