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Raynaud’s Disease and Syndrome

  • Episodic arteriolar spasm – leads to reduced blood flow in end arterioles
  • Leads to sequence of clinical features called Raynaud’s syndrome
    • Local syncope – arteriospasm causes WHITE cold palm, pain, numbness and tingling
    • Local asphyxia – arteriospam then leads to accumulation of deoxygenated blood, causing digits turn a BLUISH colour and patient feels a burning sensation
    • Local recovery – due to relief of arteriospasm, blood returns to circulation, causing a RED colour
    • Local gangrene – if spasm persists >1 hour then digits undergo ulceration and gangrene

Raynaud’s disease – primary

  • Idiopathic etiology
  • Most common in females
  • Occurs in the upper limb – usually bilateral (thumb is spared)
    • Due to arteriolar spasm as a result of abnormal sensitivity to cold
  • Peripheral pulses are present (unlike in Buerger’s disease where they are dimished)
  • Aggravated by – smoking, caffeine, oestrogen, non-selective beta blockers

Raynaud’s syndrome – secondary

  • Has known etiology, occurs secondary to a wide variety of other conditions
  • Connective tissue disorders – scleroderma, SLE, rheumatoid arthritis, Sjögren’s syndrome
  • Obstructive disorders – atherosclerosis, Buerger’s disease, thoracic outlet syndrome
  • Drugs – beta-blockers, bleomycin, bromocriptine
  • Occupation – jobs involving vibrations e.g. road drills
  • As a part of CREST syndrome

Investigations

  • Doppler US of hand
  • MR Angiography of hand
  • Digital arterial pressures
  • Nail fold capillary microscopy – to detect associated connective tissue disorders (scleroderma)
  • X-ray
  • Serology – antinuclear antibody (ANA) screening
  • Others – blood sugar, lipid profile, hypercoagulability

Treatment

  • Treat underlying cause if it is secondary
  • Avoid precipitating factors
  • Vasodilators – nifedipine, nitrates
  • Low dose aspirin – 75-100mg/day
  • ACE inhibitors/ARBs
  • Endothelin inhibitors – bosentan
    • To manage pulmonary hypertension
  • Sympatholytic drugs – prazosin (alpha-blocker)
  • Cervical sympathectomy – for non-healing digital ulcers
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