What are the signs and symptoms of vascular involvement in scleroderma?

Updated: Nov 13, 2018
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Answer

Raynaud phenomenon is part of the initial presentation in 70% of patients with systemic sclerosis; 95% eventually develop it during the course of their disease. Raynaud phenomenon may precede obvious systemic sclerosis features by months or even years.

Raynaud phenomenon that is not associated with systemic sclerosis or other autoimmune diseases is known as primary Raynaud phenomenon. It occurs in 5-15% of the general population. The female-to-male ratio is 4:1, with onset occurring usually during adolescence.

Other vascular manifestations of systemic sclerosis include the following:

  • Healed pitting ulcers in fingertips

  • Large fingertip ulcers may lead to finger amputation

  • Cutaneous and mucosal telangiectasias

  • Evidence of macrovascular involvement including non-atherosclerotic myocardial infarction


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