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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.
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.
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%).
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.
Prognosis and risk of recurrence
Severe disease may progress to digital ulcerations, necrosis, and self-amputation.
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 Society for Vascular Medicine (ESVM 2017).
1.Classification and risk stratification
Definition: classify RP into "primary RP" and "secondary RP." Avoid the termsRaynaud's syndrome" orRaynaud's disease".
Clinical history: obtain a thorough history and examination from all patients presenting with RP to ensure correct diagnosis of any underlying connective tissue disease.
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Calcium channel blockers: offer CCBs as first-line pharmacological treatment for patients with RP in whom lifestyle modification alone has failed.
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Advise lifestyle modifications such as dressing warmly, ceasing smoking, and avoiding triggers such as cold.
Obtain an occupational therapy assessment for aids if difficulties are reported.
Management of digital ulcerations: consider offering surgical management of RP in exceptional situations only, such as systemic sclerosis-related digital ulceration, given the lack of good evidence in its support.
6.Follow-up and surveillance
Indications for specialist referral
Refer patients to secondary care when there is evidence of an underlying systemic disorder, when there are signs of occlusive vascular disease, or when symptoms are severe or progressing despite lifestyle modifications and first-line drug treatment.
Refer children under age 12 to secondary care, as primary RP is less common in these age groups.