Introduction

The Raynaud phenomenon – RP – (also Reynaud’s Syndrome, Raynaud’s disease or simply Reynaud’s) is a vascular condition whereby there is excessive arterial vasoconstriction, most commonly in the hands, in response to cold temperatures or stress. Raynaud’s can also occur in the toes, ears and nose.

It can be Primary – existing without any other systemic features, or secondary – usually associated with another rheumatological disorder (e.g. Systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis or mixed connective tissue disorder). Secondary presentations account for 10-20% of cases.

It can be managed symptomatically with vasodilators (e.g. calcium channel blockers), and in cases of secondary Raynaud’s, the underlying condition should be treated.

Epidemiology

Establishing the true prevalence is difficult due to the problem in correctly classifying the disease. Estimates vary from 3-20% with a slightly higher incidence in women.

Episodes can be triggered by:

  • Cold
  • Emotional stress
  • Medications (e.g. Beta-blockers)
  • Vibration
  • Trauma
  • Smoking

Prevalence is higher in colder climates. There is often a family history.

Patients with primary Raynaud’s phenomenon are 4x more likely to have migraine.

Presentation

  • Pallor of one of more of the digits
    • Symptoms often begin in a single finger, before spreading to multiple fingers bilaterally
    • The thumb is often spared
    • Toes also commonly affected
  • Often very clear demarcation between affected areas and normal skin
  • Affected digits often then become numb or painful, followed by a period of cyanosis
  • On resolution, the digit becomes warm and red (hyperaemic) as blood flow returns
  • History
    • Primary Reynaud’s More likely to present at a younger age
    • Don’t forget to ask about associated rheumatological symptoms – such as joint pain, rash, weight loss
    • Ask about the use of vibrating machinery at home of work
    • Ask about smoking
    • Family history
    • Any medications – including over the counter medications
    • Previous history of frostbite is thought to predispose to the condition
  • Examination
    • Often normal – because very unlikely to see the patient at the time of an acute episode
    • Look for malar rash (SLE)
    • Examine any sore joints
Reynauds Phenomenon

Reynauds Phenomenon. Image from Wikimedia Commons.

Investigations

  • Often diagnosed clinically
  • Consider blood tests to help distinguish other rheumatological causes:
    • FBC, U+Es, ANA, CRP, ESR
  • Nailfold microscopy – if available – can show abnormal “capillary loops” which is suggestive of underlying rheumatological disorder

 

Diagnosis

Getting cold hands in response to cold weather is a normal physiological response. Raynaud’s occurs when this response becomes abnormal and exaggerated.

No true diagnostic criteria exist. Most specialist would agree that the condition is likely after two episodes involving colour change in the skin. True Reynaud’s colour change involves two stages:

  • Pallor – the fingers turn white
  • Cyanosis – the fingers turn blue (due to lack of oxygen)

In a young patient who is otherwise well, with normal nailfold microscopy the overwhelmingly likely cause is Primary Raynaud’s.

In older patients in particular, or if any other systemic features – take care to take a detailed history and examination to elicit an underlying pathology.  Features with a higher probably of secondary Raynaud’s include:

  • Age >30 at onset
  • Male gender
  • Complications (e.g. skin ulceration) present
  • Abnormal nailfold capillaries
  • Abnormal blood results for features associated with rheumatological disorders

 

Management

In secondary Reynaud’s, you should also treat the underlying cause, in addition to considering the management options below.

Spontaneous remission is common – and occurs in up to 60% of patients

 

Lifestyle Factors

  • Smoking cessation
  • Avoid exposure to cold
    • Consider heated mittens
    • ALWAYS wear gloves
  • Consider occupational factors – e.g. exposure to vibration
  • Avoid beta-blockers in the treatment of any co-existing condition
    • This is somewhat controversial and it is not proven that beta-blockers can cause Raynaud’s.
  • Consider moving to warmer climate in winter!

 

Medical Treatments

  • Topical nitrates
    • Fewer side effects than systemic nitrates
    • Effective at reducing the severity, but not the duration nor the frequency of attacks
  • Calcium Channel Blockers
    • Nifedipine often first line
    • Causes relaxation of vascular smooth muscle, resulting in vasodilation
    • Studies show this reduces the frequency of attacks
    • Other oral vasodilating agents have not been proven to be effective
  • IV prostaglandins – e.g. iloprost
    • Proven to reduce severity and frequency of attacks

 

Surgical Management

  • May be considered in particularly disabling cases
  • Multiple options available, including peripheral sympathectomy, arterial reconstruction

 

Specialist referral

  • Most cases can be managed in primary care
  • Consider referral if:
    • Secondary Raynaud’s
    • Children

 

Complications

  • Gangrene – sometimes with loss of the finger
  • Ulcers due to chronically ischaemic skin

 

Differentials                                                                                      

  • Cold sensitivity
    • Pain and cold sensitivity without skin colour changes
    • Occurs in 10% of the population
  • Peripheral neuropathy
    • Can cause skin changes, but will also be loss of sensational and proprioception
    • Usually an associated underlying disorder (e.g. diabetes mellitus)
  • Complex regional pain syndrome
    • Can cause skin discolouration and pain – but also often causes muscle wasting – which is NOT seen in Raynaud’s
  • Peripheral vascular disease or other occlusive event
    • Whole hand or foot, or single digit affected
    • Single occasion
    • Excruciatingly painful!
    • Needs urgent management

References

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