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Raynaud’s Phenomenon

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Overview

Raynaud’s phenomenon (RP) is characterised by paroxysmal vasospasm and subsequent vasodilation of peripheral arteries, usually in the hands and feet. It is often in response to exposure to the cold or emotional distress.

Raynaud’s phenomenon may be primary or secondary, with the latter being associated with scleroderma, other connective tissue diseases, and malignancy. 

Epidemiology

Primary Raynaud’s phenomenon:

  • More common in women
  • More common in younger women (<40 years old)
  • Associated with a family history
  • Associated with smoking
  • Associated with migraine

Secondary Raynaud’s phenomenon may be the initial manifestation of connective tissue disease:

Risk Factors

  • Female sex
  • Family history
  • Connective tissue disease
  • Vibration injury
  • Buerger’s disease
  • Smoking

Presentation

  • Digital pain and discomfort
  • Digital paraesthesia can happen as the fingers re-warm
  • Digital pallor
  • Red and/or blue discolouration of the digits
  • The discolouration is well-defined
  • Primary Raynaud’s disease tends to be bilateral and is seen in younger women (onset <40 years old)
  • Secondary Raynaud’s disease tends to be unilateral and is seen in older people (onset >40 years)

Differential Diagnoses

Physiological response to the cold

  • Normal hands can be mottled
  • RP has classic pallor that’s well-demarcated

Carpal tunnel syndrome

  • Associated conditions present: pregnancy/hypothyroidism/diabetes/rheumatoid arthritis

Cervical rib

  • May develop RP but ischaemia is often positional with the arm above the head

Investigations

If the history and physical examination suggest RP and there are no signs of a secondary cause, investigations are not necessary and RP can be clinically diagnosed.

If secondary Raynaud’s phenomenon is suspected, patients should be referred to secondary care. Other investigations would be to identify underlying connective tissue disease. Examples are:

  • Antinuclear antibody (ANA), FBC, ESR and CRP, and U&Es

Diagnosis

If the history and physical examination suggest RP and there are no signs of a secondary cause, investigations are not necessary and RP can be clinically diagnosed

A thorough history, physical examination, and laboratory testing form the workup for diagnosing secondary RP and its underlying pathology.

Management

All patients with suspected secondary Raynaud’s phenomenon should be referred to a rheumatologist.

Management of primary or mild secondary RP:

  • 1st line: supportive treatment + basic analgesia
  • 2nd line: calcium-channel blockers such as nifedipine/amlodipine/diltiazem
  • 3rd line: Angiotensin-converting enzyme (ACE) inhibitors or Angiotensin II receptor blockers (ARBs) or topical glyceryl trinitrate (GTN)
  • 4th line: alpha blockers e.g. prazosin

Management of severe secondary RP with digital ulcers or threatened digital loss:

  • 1st line: IV prostacyclin and/or PDE-5 inhibitor
  • 2nd line: surgical sympathectomy

Monitoring

  • Patients should have regular symptom reviews and physical examination
  • If underlying connective tissue is suspected, nailbed capillary microscopy should be arranged
  • Patients with abnormally dilated capillaries are at an increased risk of developing connective tissue disease e.g. scleroderma over 3-5 years

Patient Advice

  • Patients should stay warm, stop smoking, and avoid stress

Complications

  • Cellulitis
  • Osteomyelitis
  • Threatened ischaemic digit
  • Ischaemic digital ulcers
  • Autoamputation
  • Necrosis with gangrene

Prognosis

  • Primary RP may go into remission
  • An underlying disorder develops in around 13% of patients
  • Secondary cases tend to be more problematic with ulceration, scarring, or gangrene

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