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Updated: May 22 2021

Raynaud's Syndrome

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https://upload.orthobullets.com/topic/6098/images/white discoloration.jpg
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https://upload.orthobullets.com/topic/6098/images/gangrene.jpg
  • Introduction
    • Raynaud's is an exaggerated vasocontriction of the digital arteries in response to a variety of stimuli. It takes two forms
      • Raynaud's Phenomenon or Raynaud's Syndrome
        • vasospastic disease with a known underlying cause
      • Raynaud's Disease
        • vasospastic disease with no known cause (idiopathic)
  • Etiology
    • Pathophysiology
      • periodic digital ischemia due vasoconstriction induced by
        • cold temperature or
        • sympathetic stimuli including pain or emotional stress
      • triphasic color change (white-blue-red progression)
        • digits turn white from vasospasm and interruption of blood flow
        • blue discoloration follows from cyanosis and venous stasis
        • finally digits turn red as a result of rebound hyperemia
        • dysesthesias often follow color changes
  • Raynaud's Phenomenon
    • Vasospastic disease with a known underlying disease
      • epidemiology
        • demographics
          • occasional female predominance
          • age >40 years (generally older than patients with Raynaud's disease)
        • location
          • affects the distal aspect of digits
      • associated conditions
        • connective tissue disease
          • scleroderma (80-90% incidence of Raynaud's phenomenon)
          • SLE (18-26%)
          • dermatomyositis (30%)
          • RA (11%)
          • CREST syndrome
            • calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias
          • neurovascular compression (thoracic outlet syndrome)
    • Presentation
      • Symptoms
        • asymmetric findings
        • rapid progression
      • Physical exam
        • peripheral pulses often absent
        • frequent trophic skin changes (including ulceration and gangrene)
        • abnormal Allen test
    • Studies
      • labs
        • blood chemistry - often abnormal
      • invasive studies
        • microangiology - often abnormal
        • angiography - often abnormal
    • Imaging
      • radiographs
        • normal and not indicated
  • Raynaud's Disease
    • Vasospastic disease with no known cause (idiopathic)
      • epidemiology
        • seen in young premenopausal women (age <40 years)
    • Presentation
      • symptoms
        • often bilateral
        • slow progression
      • Allen and Brown Criteria for Raynaud's Disease
        • Intermittent attacks with discoloration of acral parts
        • Bilateral involvement
        • Absence of clinical arterial occlusion
        • Gangrene and trophic changes are rare
        • Symptoms present for >2 years
        • Predominance in women
        • Absence of other disease to explain findings
      • physical exam
        • peripheral pulses usually present
        • trophic skin changes are uncommon
        • normal Allen test
    • Imaging
      • radiographs
        • normal and not indicated
    • Studies
      • labs usually normal
      • invasive studies usually normal
      • diagnosis
        • based on Allen and Brown criteria
  • Differential 
    • Factors to differentiate Raynaud's Phenomenon or Raynaud's Disease
      • age
      • gender
      • time frame of symptom progression
      • severity of exam findings
  • Treatment
    • Nonoperative
      • medical management & lifestyle modifications
        • indications
          • first line of treatment
        • modalities
          • smoking cessation and avoidance of cold exposure is critical
          • thermal biofeedback techniques
          • medications include
            • topical nitrates
            • calcium channel blockers
            • ASA
            • intra-arterial reserpine
            • dipyridamole (Persantine)
            • pentoxifylline (Trental)
            • Sildenafil
      • Botulinum toxin A injections
        • indications
          • medical management has failed
          • presence of ischemia and pain
          • ulcerations not a contraindication
          • note this is "off-label" use
        • Shown to improve digital perfusion
    • Operative
      • thorascopic sympathectomy
        • indications
          • fallen out of favor due to rebound hypersympathetic response
      • digital sympathectomy
        • indications
          • severe cases that fail conservative treatment (impending gangrene)
          • periarterial sympathectomy most common method
            • removal of adventitia and sympathetic nerve fibers
            • may extend to forearm
          • sympathectomy is not indicated calcific arterial disease
      • microvascular reconstruction
        • indications
          • may be indicated in rare situations with occlusion of small segment of one major artery while other is spared/minimal involvement
  • Complications
    • digital ischemia leading to ulcerations and/or gangrene
      • may necessitate amputation of digit(s)
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