• Raynaud's phenomenon refers to the symptoms of digital ischaemia
• 80% of patients are women
• Commonest before the age of 35 years
• Population prevalence may be as high as 5%
• Most patients have primary disease
• Primary Raynaud's disease occurs in patients with normal arteries
• Symptoms are due to an abnormal reversible physiological response
• Secondary Raynaud's disease occurs in patients with underlying systemic disorder

Primary Raynaud's disease

• Due to excessive vasoconstriction of digital arteries
• Vessels are normal between episodes
• Cooling of the hands results in intense vasoconstriction
• Flow in the digital arteries ceases at the critical closing temperature
• Reopening of blood vessels requires a rise in perfusion pressure
• Possible pathophysiological mechanisms include
  • Increased sympathetic activity
  • Increased sensitivity to adrenergic stimuli
  • Increased number of alpha-receptors in the vessel wall

Cold provocation test

Secondary Raynaud's disease

• Due to conditions associated with
  • Abnormal vessel walls
  • Increased blood viscosity
• Connective tissue disease
  • Scleroderma
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Dermatomyositis
  • Polyarteritis nodosa
• Haematological
  • Cryoglobulinaemia
  • Cold haemagglutinaemia
  • Paraproteinaemia
  • Thromboembolic disease
• Arterial
  • Buerger's disease
  • Atherosclerosis
  • Arteritis
• Neurovascular
  • Thoracic outlet syndrome
  • Carpal tunnel syndrome
  • Crutch pressure
• Drugs
  • Nicotine
  • Beta-blockers
  • Ergot derivatives
  • Sympathomimetics
• Miscellaneous
  • Vibration white finger
  • Neoplasms
  • Poliomyelitis
  • Hypothyroidism
  • Chronic renal failure

Clinical features

• Diagnosis can usually be made from clinical history
• Consists of a triphasic response provoked by exposure to cold
• Phase 1 - pallor due to intense vasoconstriction
• Phase 2 - cyanosis due to desaturation of haemoglobin
• Phase 3 - erythema due to hyperaemia and restoration of circulation
• Primary disease is usually bilateral, symmetrical and involves all fingers
• Secondary disease is usually patchy and asymmetrical
• Symptoms are often milder in primary disease
• Examination often shows peripheral pulses to be normal
• Features of thoracic outlet syndrome or connective tissue disorders may be present

Picture provided by Eldeeb Mabrouk, University Hospital, Alexandria, Egypt

Investigations

• Should be guided by clinical features
• Serology should include - FBC, ESR, anti-nuclear antibodies
• Electrophoresis, cold agglutinins and fibrinogen levels may identify hyperviscosity states
• Chest x-ray and thoracic outlet views may show cervical rib
• Duplex ultrasound or arteriography may be indicated is suspicion of arterial disease
• No reliable method of provoking Raynaud's phenomenon exists  
• Raynaud's phenomena is seen in
  • 90% patients with scleroderma
  • 30% pateinst with rheumatoid arthritis
  • 30% patients with primary Sjogren's syndrome
  • 10% patients with rheumatoid arthritis

Treatment options

• Prevention
  • Clothing - wearing of thermal fabrics
  • The use of hand warmers or electric gloves
  • Stop smoking
  • Change job if vibration induces
  • Avoid sympathetic stimulants
• Topical agents
  • GTN paste
  • Prostaglandin analogues
• Oral agents
  • Sympatholytic agents - reserpine, guanethidine
  • Alpha-blockers -  phenoxybenzamine, prazocin
  • Beta-stimulants - terbutaline
  • Vasodilators - nifedipine, diltiazen
  • Fibrinolytic agents - pentoxifylline
  • Serotonin antagonists - ketanserin
• Invasive
  • Intraarterial reserpine
  • Intravenous iloprost
• Surgery
  • Thoracic outlet surgery
  • Embolectomy
  • Sympathectomy

Bibliography

Block J A,  Sequeira W.  Raynaud's phenomenon.  Lancet 2001;  357:  2042-2048.