How to manage Raynaud's phenomenon?

Management of Raynaud's Phenomenon

Start with cold avoidance and smoking cessation for all patients, then use nifedipine (or another dihydropyridine calcium channel blocker) as first-line pharmacotherapy, escalating to phosphodiesterase-5 inhibitors for inadequate response, and reserve intravenous prostacyclin analogues for severe refractory disease. 1, 2

Non-Pharmacological Management (Essential for All Patients)

Trigger Avoidance

• Avoid cold exposure by wearing proper warm clothing including coat, mittens (not gloves), hat, dry insulated footwear, and hand/foot warmers 3, 1
• Avoid direct contact with cold surfaces and thoroughly dry skin after moisture exposure 4
• Smoking cessation is mandatory as tobacco worsens vasospasm 3, 1, 2
• Avoid vibration injury, repetitive hand trauma, and emotional stress 3, 1
• Discontinue or avoid drugs that worsen Raynaud's: beta-blockers, ergot alkaloids, bleomycin, and clonidine 3, 1, 2

Physical Therapy and Exercise

• Physical exercise should be considered to improve hand function and stimulate blood flow 3, 4
• Teach patients exercises to generate heat and prevent symptom onset 3, 1

Patient Education

• Offer patient education and self-management support to improve hand function, quality of life, and ability to perform daily activities 3, 1

Pharmacological Management Algorithm

First-Line: Calcium Channel Blockers

Nifedipine (dihydropyridine-type) is the first-line pharmacotherapy due to clinical benefit, low cost, and acceptable adverse effects 1, 2

• Meta-analyses confirm nifedipine reduces both frequency and severity of Raynaud's attacks 1
• If nifedipine is poorly tolerated or ineffective, consider other dihydropyridine calcium channel blockers 1
• Common adverse effects include hypotension, peripheral edema, and headaches 5

Second-Line: Phosphodiesterase-5 Inhibitors

Use sildenafil or tadalafil when calcium channel blockers provide inadequate response 1, 2

• PDE5 inhibitors effectively reduce frequency and severity of attacks 1, 2
• These agents are also effective for both healing and prevention of digital ulcers, making them particularly valuable in secondary Raynaud's 1, 2
• Cost and off-label use may limit utilization 1

Third-Line: Intravenous Prostacyclin Analogues

Intravenous iloprost should be considered for severe Raynaud's unresponsive to oral therapies 3, 1, 2

• Iloprost has demonstrated efficacy in reducing frequency and severity of attacks 1, 6
• Most promising drug for management of secondary Raynaud's disease 6

Management of Digital Ulcers (Secondary Raynaud's)

Prevention of New Digital Ulcers

• Bosentan (endothelin receptor antagonist) is effective for preventing new digital ulcers, particularly in systemic sclerosis patients with multiple ulcers 3, 1, 2
• PDE5 inhibitors can prevent digital ulcers 1, 2
• Prostacyclin analogues can prevent digital ulcers 1

Healing of Existing Digital Ulcers

• Intravenous iloprost has proven efficacy for healing existing digital ulcers 1, 2
• PDE5 inhibitors improve healing of digital ulcers 1, 2
• Note: Bosentan prevents new ulcers but does not enhance healing 3, 5

Treatment Stratification by Severity

Mild Raynaud's (Primary)

• Non-pharmacological measures alone may suffice 1, 2
• Add calcium channel blockers if symptoms affect quality of life 1

Moderate to Severe Raynaud's

• Calcium channel blockers as first-line 1, 2
• Add or switch to PDE5 inhibitors for inadequate response 1, 2

Severe Refractory Raynaud's

• Intravenous prostacyclin analogues for frequent attacks despite above treatments 1, 2
• Consider digital sympathectomy in severe cases with critical ischemia 3, 7

Critical Pitfalls to Avoid

• Missing secondary causes (especially systemic sclerosis) leads to delayed treatment and poor outcomes including digital ulcers, gangrene, and potential amputation 1, 2
• Delaying pharmacotherapy in secondary Raynaud's increases complication risk 2
• Using beta-blockers or other vasoconstrictive drugs can induce or worsen symptoms 1, 2

Monitoring and Follow-Up

• Reassess treatment efficacy at 3-6 months and escalate therapy if inadequate response 2
• Regular monitoring for digital ulcers, gangrene, and progression of underlying disease is essential in secondary Raynaud's 2, 4
• Most pharmacological treatments reduce severity and frequency of attacks but do not completely abolish vasospastic episodes 6