What causes Raynaud phenomenon in CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Microvascular abnormalities and dysfunction are central to the pathogenesis of scleroderma-associated Raynaud phenomenon.

Endothelial injury is believed to result in intimal hyperplasia and fibrosis, concentric narrowing of digital arteries by as much as 75-80%, and occlusion by intravascular thrombi.

Resting blood flow in fingers was lower in scleroderma patients compared with normal controls as measured by laser Doppler flowmeter. Arteries from scleroderma patients have significantly increased sensitivity to alpha2-adenoreceptor–mediated vasoconstriction. Whether this is a consequence or cause of endothelial cell injury and dysfunction is unclear.

Endothelin, a naturally occurring peptide, has been implicated as a pathologic mediator of vasoconstriction, fibrosis, vascular hypertrophy, and inflammation in patients with Raynaud syndrome. [29]

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