- 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.
|