management of Raynaud's disease/phenomenon

Last edited 12/2021 and last reviewed 12/2021

The first step in management of Raynaud’s phenomenon is lifestyle modification. Majority will respond to conservative measures and need no further treatment but patients suspected of having an underlying cause should be referred to secondary care (1).

For patients who do not respond to conservative measures, various groups of drugs have been used in the management. These should be used on a case by case basis:

vasodilators

  • calcium channel blockers
    • is the only drug licensed for use in Raynaud’s phenomenon
    • considered to be the drugs of choice (2)
    • non-cardioselective dihydropyridine calcium channel blockers are used commonly
      • nifedipine is useful for reducing the severity and frequency of vasospastic attacks
      • an initial dosage of 10mg once daily is suggested. This dose is increased to 10mg twice daily after a week and thereafter increased gradually to 20mg twice daily. The maximum dosage (if tolerated) is 20mg three times daily (1)
      • effects may be short lived and patients commonly report  side-effects such as headache, dizziness, flushing and ankle swelling - the side effects associated with nifedipine use can be reduced by using the slow-release preparation rather than the standard capsules (1)
  • topical nitrates
    • large randomized control trials have reported that topical nitrates applied to the dorsum of the finger reduces the severity of Raynaud’s phenomenon, but not the duration or frequency of attacks
  • prostaglandins
    • the European League Against Rheumatism recommends prostaglandins in patients who do not have any improvement  with calcium channel blockers
    • intravenous ilopros has shown to reduces the frequency and severity of attacks
  • phosphodiesterase type 5 inhibitors (sildenafil, tadalafil, and vardenafil)
    • oral sildenafil has been reported to decrease in the frequency and severity of attacks

Inhibitors of vasoconstriction (1)

  • angiotensin receptor antagonists
    • trials have shown that losartan reduces the frequency and severity of attacks to a greater extent than nifedipine but  there is lack of sufficient evidence
  • endothelin receptor antagonists (bosentan)
    • the European League Against Rheumatism recommends in patients with refractory symptoms to treatment with calcium channel blockers and prostaglandins.
  • serotonin reuptake inhibitors
    • a pilot study of 53 patients showed that fluoxetine reduces the severity and frequency of attacks compared with nifedipine in primary Raynaud’s phenomenon.

Surgical interventions can be considered in certain patients with severe and disabling symptoms. These include:

  • arterial reconstruction
  • peripheral sympathectomy
  • embolectomy
  • ulcer debridement
  • a combination of techniques (1)

A systematic review was undertaken regarding vasodilators in the management of Raynaud's phenomenon. The systematic review considered calcium channel blockers as the drug of choice in this condition and therefore considered alternative vasodilator agents to calcium channel blockers in the management of this condition (2). The systematic review considered:

  • alpha blockers
  • prostaglandin/prostacyclin analogues
  • thromboxane synthase inhibitors
  • selective serotonin reuptake inhibitors
  • nitrate/nitrate derivatives
  • phosphodiesterase inhibitors

This updated review (15 studies [7 new]; n=635) found the evidence is insufficient to support use of vasodilators; they may even worsen disease (2)

Note:

  • since many serious conditions are associated with secondary Raynaud's phenomenon, the following groups should be referred to a specialist (rheumatologist)
    • the diagnosis is in doubt
    • a secondary cause is suspected
    • the cause is thought to be job related (refer to occupational health services)
    • the patient is aged under 12 years
    • digital ulcerations are present
    • the symptoms are poorly controlled, despite appropriate conservative management.

 Reference: