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Raynaud's phenomenon

Background

Overview

Definition
RP is a vasospastic disorder that results in characteristic, triphasic changes in skin color occurring in response to various external triggers, including exposure to cold.
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Pathophysiology
RP may be idiopathic in origin (primary RP), or occur in the context of connective tissue diseases (secondary RP), such as systemic sclerosis, dermatomyositis, SLE, Sjögren's syndrome, mixed connective tissue disease, or undifferentiated connective tissue disease.
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Epidemiology
In the US, the prevalence of primary RP is estimated at 4.85% (95% CI, 2.08-8.71%), while the pooled annual incidence is estimated at 0.25% (95% CI, 0.19-0.32%).
1
Disease course
In patients with RP, exposure to cold triggers intense sympathetic vasoconstriction in skin areas bearing specialized arterio-venous anastomoses that normally contribute to thermoregulation (fingers, toes, nose, and ear tip). In patients with secondary RP, abnormal endothelial function increases the risk of ischemic complications.
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Prognosis and risk of recurrence
Severe disease may progress to digital ulcerations, necrosis, and self-amputation.
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Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Raynaud's phenomenon are prepared by our editorial team based on guidelines from the European League Against Rheumatism (EULAR 2017) and the European Society for Vascular Medicine (ESVM 2017). ...
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Classification and risk stratification

Definition
As per ESVM 2017 guidelines:
Classify RP into primary RP and secondary RP. Avoid using terms Raynaud's syndrome or Raynaud's disease.
B
Classify conditions associated with RP into the following groups:
true associated disorders with etiological links
conditions worsening RP or precipitating its appearance
conditions not causing vasospasm but digital necrosis
B
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Diagnostic investigations

History and physical examination: as per ESVM 2017 guidelines, elicit a thorough history and perform a physical examination in all patients presenting with RP to ensure correct diagnosis of any underlying connective tissue disease.
B

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  • Capillaroscopy

  • Laboratory evaluation

Medical management

CCBs
As per ESVM 2017 guidelines:
Offer CCBs as first-line pharmacotherapy in patients with RP if lifestyle modification alone has failed.
A
Use slow-release nifedipine and increase dose gradually to minimize vasodilatory side effects. Encourage patients to tolerate minor side effects for 2-3 weeks, as they may subside with time.
B

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  • PDE5 inhibitors

  • Iloprost

  • Fluoxetine

Nonpharmacologic interventions

Lifestyle modifications
As per ESVM 2017 guidelines:
Advise lifestyle modifications, such as dressing warmly, smoking cessation, and avoiding triggers such as cold.
B
Obtain an occupational therapy assessment for aids if difficulties are reported.
B

Surgical interventions

Management of digital ulcerations: as per ESVM 2017 guidelines, consider offering surgical management of RP in exceptional situations, such as systemic sclerosis-related digital ulceration, given the lack of good evidence in its support.
C

Specific circumstances

Patients with systemic sclerosis-associated RP: as per EULAR 2017 guidelines, consider offering dihydropiridine CCBs, usually oral nifedipine, as first-line therapy for systemic sclerosis-associated RP.
B
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More topics in this section

  • Patients with systemic sclerosis-associated digital ulcerations (pharmacotherapy)

  • Patients with systemic sclerosis-associated digital ulcerations (surgical management)

Follow-up and surveillance

Indications for specialist referral
As per ESVM 2017 guidelines:
Refer patients to secondary care when there is evidence of an underlying systemic disorder, signs of occlusive vascular disease, or severe or progressing symptoms despite lifestyle modifications and first-line pharmacotherapy.
B
Refer pediatric patients aged < 12 years to secondary care, as primary RP is less common in these age groups.
B