Vasospastic disorders (Raynaud’s phenomenon)

Overview:

Raynaud’s phenomenon (RP):

• Is the general term which describes the clinical features of episodic digital vasospasm in the absence of an identifiable associated disorder

Secondary Raynaud’s syndrome (RS):

• This is when the phenomenon occurs secondary to one of the conditions listed below

Conditions associated with Raynaud’s syndrome:

Connective tissue disorders:

• Systemic sclerosis 90%
• Systemic lupus erythematosus (SLE) 30%
• Mixed connective tissue disease 80%
• Dermatomyositis/polymyositis 30%

Macrovascular disease:

• Thoracic outlet obstruction
• Atherosclerosis
• Buerger’s disease
• Radiation arteritis

Occupational trauma:

• Vibration white finger (VWF)
• Chemical exposure (e.g. nitrates, polyvinyl chloride)
• Repeated exposure to extreme cold

Drugs:

• Cytotoxic drugs
• Ergotamine
• Beta-blockers
• Cyclosporine

Miscellaneous:

• Malignancy
• Arteriovenous fistula

Epidemiology and aetiology:

• 10x commoner in females than males
• Affects (in a mild form) up to 25% of the young female population

Risk factors include:

• OCP
• Some migraine drugs
• Tobacco

Clinical features:

There are 3 phases:

• Pallor – because of digital artery spasm
• Cyanosis – from the accumulation of deoxygenated blood
• Redness (rubor) – reactive hyperaemia as blood flow returns

Pain is usual unless there are other complications (e.g. digital ulceration and gangrene)

Diagnosis:

• In the majority, the diagnosis of RP can be made on symptoms and physical findings; additional investigations are not required unless secondary RS is suspected
• Only a minority have clear evidence of connective tissue disease at presentation
• Presentation for the first time in childhood or over the age of 30 increases the likelihood of RS
• 80% of those who present >60 years of age have an underlying disorder (although it is most likely atherosclerosis)

Medical management:

• Most often, reassurance about the usually benign nature of their condition, advice to stop smoking and to avoid exposure to cold are sufficient
• Numerous drugs have been used, the best of which appears to be the calcium-channel blocker nifedipine, although side-effects are common
• Vasodilators may also be useful

In those with severe attacks:

Admission to hospital for a 5-day infusion of prostacyclin may provide great symptomatic relief in the winter months and, for unknown reasons, the beneficial effects may last up to 6 weeks

Surgical management:

Secondary RS caused by macrovascular arterial disease is nearly always unilateral and may progress rapidly to tissue loss in the hand if the underlying lesion is not identified and treated

Sympathectomy is useful in the long-term treatment of RS in the feet, but NOT in the hand

In the variant CREST syndrome, digits affected by severe ulceration or calcium deposits may require amputation