CKS Clinical Solutions - Raynaud's phenomenon

The case

Pallor of the fingers is an indicator of Raynaud's phenomenon
Pallor of the fingers is an indicator of Raynaud's phenomenon

A 40-year-old woman complains of her hands becoming painful and changing colour, especially during the winter months.

What is Raynaud's phenomenon?
Raynaud's phenomenon is episodic vasospasm of the arteries in the extremities, causing pallor followed by cyanosis and/or redness of the fingers and toes.1

Primary Raynaud's phenomenon occurs without an underlying cause.1 Secondary Raynaud's phenomenon occurs in association with an underlying disease (such as scleroderma, systemic lupus erythematosus, rheumatoid arthritis).1 Primary Raynaud's phenomenon is more common in women than men,2 and onset is usually in the second or third decade.2-4

How should I diagnose Raynaud's phenomenon?
Diagnose Raynaud's phenomenon if there is a history of clearly demarcated pallor of the digits, followed by cyanosis and/or erythema. Symptoms are usually precipitated by cold.

Distinguish from other conditions such as chilblains or reflex sympathetic dystrophy.

  • How should I differentiate between primary and secondary Raynaud's phenomenon?

Diagnose primary Raynaud's phenomenon if all the following criteria are met:

  • Symmetrical episodes.
  • No tissue necrosis, ulceration, gangrene or severe ischaemia.
  • Normal nail-fold capillaries.
  • Normal ESR.
  • Negative antinuclear antibodies.

Suspect secondary Raynaud's phenomenon if any of the following are present:

  • Onset over 30 years of age.
  • Episodes are asymmetrical.
  • Underlying disease.
  • Positive antinuclear antibodies.
  • Abnormal nail-fold capillaries (difficult to detect).
  • Digital ulcers, infection, gangrene or severe ischaemia.
  • When should I refer?

Refer anyone with severe ischaemia of one or more digits; if the diagnosis is in doubt; if an underlying cause is suspected or for poorly controlled symptoms despite treatment.

  • How should I manage Raynaud's phenomenon?

Advise the woman to keep her whole body warm, minimise stress and stop smoking.

If these fail, offer a trial of nifedipine: either immediate-release up to 20mg three times daily, or modified-release up to 60mg once daily. If nifedipine is not tolerated, consider nicardipine, amlodipine or felodipine.

Evidence
Assessment recommendations are based on expert opinion from a number of published review articles.1-7 All but one review1 advised antinuclear antibodies should be checked routinely in all people with Raynaud's phenomenon. An update for GPs8 suggests referral to a specialist if secondary Raynaud's is suspected. The recommendation to offer nifedipine is based on a systematic review9, and two BMJ reviews1,10 show nifedipine to reduce severity of attacks.

REFERENCES
1. Pope J. (2007a) Raynaud's phenomenon (primary). Clin Evid. BMJ Publishing Ltd.

2. Planchon B, Pistorius M, Beurrier P et al. Angiology, 1994; 45(8): 677-86.

3. Nigrovic P, Fuhlbrigge R Sundel R. Pediatrics, 2003; 111(4): 715-21.

4. Suter L, Murabito J, Felson D et al. Arthritis Rheum, 2005; 52(4): 1259-63.

5. LeRoy E and Medsger T. Clin Exp Rheumatol, 1992; 10(5): 485-8.

6. Wigley FM. N Engl J Med 2002; 347(13): 1001-8.

7. Bakst R, Merola J, Franks A et al.J Am Acad Dermatol, 2008; 59(4): 633-53.

8. Raynaud's & Scleroderma Association (2008). Raynaud's & Scleroderma: An Update for GPs.

9. Thompson AE and Pope JE. Rheumatology, 2005; 44(2), 145-50.

10. Herrick A. (2008a) Raynaud's phenomenon (secondary). Clin Evid. BMJ Publishing Ltd.

Reliable, evidence-based answers to real-life clinical questions, from the NHS CKS service. CKS is part of the National Library for Health.

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