What are the guideline sources, guidelines and references for Raynaud_s phenomenon ?
Guideline sources The following summarized guidelines for the evaluation and management of Raynaud s phenomenon (RP) are prepared by our editorial team based on guidelines from the European Society for Vascular Medicine (ESVM 2017). 1 2 2 3 3 4 5 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. 2 Epidemiology 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, Sj gren's syndrome, mixed connective tissue disease, or undifferentiated connective tissue disease. 3 Pathophysiology In the United States, 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%). 2 Disease course https://web.pathway.md/diseases/recGtfQmVsXezer5w 1/3 6/24/23, 2:57 PM Raynaud's phenomenon Pathway 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. 3 4 Prognosis and risk of recurrence Severe disease may progress to digital ulcerations, necrosis, and self-amputation. 5 Pathway Raynaud's phenomenon Investigation and management Guidelines 1. Classification and risk stratification Definition: classify RP into "primary RP" and "secondary RP." Avoid the terms "Raynaud's syndrome" or "Raynaud's disease". B 2. Diagnostic investigations Clinical history: obtain a thorough history and examination from all patients presenting with RP to ensure correct diagnosis of any underlying connective tissue disease. B Laboratory evaluation: obtain basic laboratory testing (CBC, antinuclear antibodies, ESR or CRP) in all patients presenting with RP. B Capillaroscopy: Obtain capillaroscopy, when available, in all patients presenting with RP. B Ensure capillaroscopy is carried out using equipment of good optical quality and by an experienced operator, usually in secondary or tertiary care. B 3. Medical management Calcium channel blockers: offer CCBs as first-line pharmacological treatment for patients with RP in whom lifestyle modification alone has failed. A Show 3 more 4. Nonpharmacologic interventions https://web.pathway.md/diseases/recGtfQmVsXezer5w 2/3 6/24/23, 2:57 PM Raynaud's phenomenon Pathway Lifestyle modifications: Advise lifestyle modifications such as dressing warmly, ceasing smoking, and avoiding triggers such as cold. B Obtain an occupational therapy assessment for aids if difficulties are reported. B 5. Surgical interventions 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. C 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. B Refer children under age 12 to secondary care, as primary RP is less common in these age groups. B References 1. Belch J, Carlizza A, Carpentier PH et al. ESVM guidelines - the diagnosis and management of Raynaud's phenomenon. Vasa. 2017 Oct;46 6 413 423. Open 2. Garner R, Kumari R, Lanyon P et al. Prevalence, risk factors and associations of primary Raynaud's phenomenon: systematic review and meta-analysis of observational studies. BMJ Open. 2015 Mar 16;5 3):e006389. Open 3. Wigley FM, Flavahan NA. Raynaud's Phenomenon. N Engl J Med. 2016 Aug 11;375 6 556 65. Open 4. Flavahan NA. A vascular mechanistic approach to understanding Raynaud phenomenon. Nat Rev Rheumatol. 2015 Mar;11 3 146 58. Open 5. Hughes M, Herrick AL. Raynaud's phenomenon. Best Pract Res Clin Rheumatol. 2016 Feb;30 1 112 32. Open 6. Patricia Senet, Herv Maillard, Elisabeth Diot et al. Efficacy and Safety of Botulinum Toxin in Adults with Raynaud's Phenomenon Secondary to Systemic Sclerosis: A Multicenter, Randomized, Double-Blind, Placebo-Controlled Study. Arthritis Rheumatol. 2023 Mar;75 3 459 467. Open https://web.pathway.md/diseases/recGtfQmVsXezer5w 3/3