Updated: 6/10/2019

Raynaud's Syndrome

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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)
  • 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
  • Imaging
    • radiographs
      • normal and not indicated 
  • Studies
    • labs
      • blood chemistry - often abnormal
    • invasive studies
      • microangiology - often abnormal
      • angiography - often abnormal 
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
    • 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
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
  • Absence of other disease to explain findings
  • Predominance in women
Differential Diagnosis
  • 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
        • 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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