Raynaud's phenomenon

Episodic pallor or cyanosis of the fingers caused by vasoconstriction in response to cold or emotional stress

• Starts as sudden-onset pallor, followed by cyanosis as the capillary blood becomes desaturated. The attack subsides with the return of arterial inflow, and post-ischaemic vasodilation results in hyperaemia and rubor of the skin
• Toes can also be affected, but much more often the fingers
• Refer to primary Raynaud's as Raynaud's Disease, and secondary Raynaud's as Raynaud's Phenomenon. Or, you can refer to them all as Raynaud's Syndrome.

Primary vs secondary

• Primary RP (most common)
  • Benign process, not associated with any structural vascular change
  • Pathogenesis unknown
  • Gangrene or tissue necrosis is never a/w primary RP
  • Nail fold capillary exam, ESR, and serologic markers, are normal
• Secondary - associated with an underlying disease process
  • When the artery is narrowed because of other large or small vessel disease, there is a lower critical closing pressure, and a relatively normal vasoconstrictor response with result in temporary closure of the vessel
  • Can progress to digital ulceration, scarring or gangrene
  • Aetiology

Both primary and secondary disease:

Clinical findings

• Typically, attacks of pallor involving part or all of one or many fingers, brought on by cold exposure with full and rapid recovery on rewarming of the digits
• Episodes self-limited, lasting 10-20 minutes
• Thumbs are frequently spared - if involved, suggests secondary Raynaud's
• Can involve toes too
• Can occur several times a day to several times a week
• Pain is not usually a feature of the pallor or cyanotic phase - patients with secondary RP are more likely to complain of digital pain on rewarming
• Examination
  • Signs of autoimmune disease - skin thinning/tightening, sclerodactyly, telangiectasis
  • Ulceration or hyperkeratotic areas on fingers suggesting healed ulceration
  • Splinter haemorrhages
  • Pulse evaluation including Allen test

Investigation

• DUS
• ANA (for CTD such as scleroderma) and rheumatoid factor (for RA)

Treatment

• Non-pharmacologic
  • Avoid cold/mittens
  • Avoid caffeine/smoking
• Pharmacologic
  • Indicated in patients who have failed conservative therapy
  • First-line - long-acting CCB - nifedipine 30mg/day up to 120 mg/day (start at 10mg TDS). Results in about 50% reduction in frequency and severity of attacks.
• Surgical