What is the management and treatment for Raynaud's (Raynaud's disease) symptoms?

Management of Raynaud's Phenomenon

All patients with Raynaud's phenomenon should begin with trigger avoidance and lifestyle modifications, followed by nifedipine as first-line pharmacotherapy if symptoms affect quality of life, with escalation to phosphodiesterase-5 inhibitors for inadequate response, and intravenous prostacyclin analogues reserved for severe refractory disease. 1, 2

Initial Assessment Priority

Before initiating any treatment, always evaluate for systemic sclerosis and other connective tissue diseases, as delayed diagnosis leads to digital ulcers and poor outcomes. 2 Look specifically for:

• Severe, painful episodes with digital ulceration (red flag for secondary Raynaud's) 1
• Systemic symptoms: joint pain, skin changes, dysphagia, weight loss, malaise, fatigue, photosensitivity, dry eyes, dry mouth 3
• Age at onset: older age suggests secondary disease 4
• Pattern of involvement: entire hand involvement rather than individual digits suggests secondary Raynaud's 3

Non-Pharmacological Management (Mandatory for All Patients)

These measures must be implemented before or alongside any pharmacotherapy: 2

• Cold avoidance: wear proper warm clothing including coat, mittens (not gloves), hat, insulated footwear, and use hand/foot warmers 1, 2
• Smoking cessation is mandatory as smoking directly worsens vasospasm 2, 5
• Discontinue triggering medications: beta-blockers, ergot alkaloids, bleomycin, clonidine 1, 2
• Stress management techniques to reduce attack frequency 2
• Avoid vibration injury and repetitive hand trauma, particularly in occupational settings 2
• Physical therapy with exercises to generate heat and stimulate blood flow 1, 2

Critical pitfall: Continuing triggering medications like beta-blockers will undermine all treatment efforts. 2

Pharmacological Treatment Algorithm

First-Line: Calcium Channel Blockers

Nifedipine (dihydropyridine-type calcium channel blocker) is first-line pharmacotherapy for both primary and secondary Raynaud's, reducing both frequency and severity of attacks with acceptable adverse effects and low cost. 1, 2 Meta-analyses of randomized controlled trials confirm efficacy. 1

• Typical dosing: Extended-release nifedipine 30 mg at bedtime 5
• Expected response: 70-80% respond with decreased severity and frequency, but 20-50% develop intolerable side effects 5
• Adverse effects: hypotension, vasodilatation, peripheral edema, headaches 4
• Alternative: Other dihydropyridine-type calcium channel blockers can be considered if nifedipine lacks benefit or is poorly tolerated 1

Second-Line: Phosphodiesterase-5 Inhibitors

For inadequate response to calcium channel blockers, add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil), which effectively reduce frequency and severity of Raynaud's attacks. 1, 2 These agents are also effective for both healing and prevention of digital ulcers, though prevention results are mixed. 1

• Limitation: Cost and off-label use may limit utilization 1

Third-Line: Intravenous Prostacyclin Analogues

For severe Raynaud's unresponsive to oral therapies, use intravenous iloprost (prostacyclin analogue), which has demonstrated efficacy in reducing frequency and severity of attacks. 1, 2 Iloprost is the most promising drug in management of secondary Raynaud's disease. 6

Management of Digital Ulcers

Prevention of New Digital Ulcers

Bosentan (endothelin receptor antagonist) is effective for preventing new digital ulcers, particularly in systemic sclerosis patients with multiple existing ulcers. 1, 2 Two large studies demonstrate that bosentan reduces the number of new digital ulcers, though it does not affect healing period. 4

• Alternative prevention options: Phosphodiesterase-5 inhibitors and prostacyclin analogues 1

Healing of Existing Digital Ulcers

For healing digital ulcers, use intravenous iloprost or phosphodiesterase-5 inhibitors, both with proven efficacy. 1, 2

• Local wound care: soap-and-water washes with either damp dressing or Silvadene cream 5
• Adjunctive therapy: pentoxifylline and antibiotics as needed 5
• Severe cases: If chronic non-healing or intractable pain despite medical therapy, fingertip amputation may be required, though healing is slow 5

Treatment Algorithm by Severity

Mild Raynaud's (Primary)

• Non-pharmacological measures alone may suffice 1
• Add nifedipine if symptoms affect quality of life 1

Moderate to Severe Raynaud's or Inadequate Response

• Add or switch to phosphodiesterase-5 inhibitors 1

Severe Refractory Raynaud's

• Consider intravenous prostacyclin analogues 1
• Critical pitfall: Delaying escalation in secondary Raynaud's leads to digital ulcers and poor outcomes; more aggressive therapy is required 2

Digital Ulcers Present

• Prevention: bosentan, phosphodiesterase-5 inhibitors, or prostacyclin analogues 1
• Healing: intravenous iloprost or phosphodiesterase-5 inhibitors 1

Additional Pharmacological Options (Limited Evidence)

• Fluoxetine (SSRI): might be considered for Raynaud's attacks, though evidence is limited to small studies 1
• Topical nitrates: can be used but limited by adverse effects including flushing, headache, and hypotension 4
• Minimal evidence: biofeedback, acupuncture, ceramic-impregnated gloves, antioxidants, essential fatty acids, Ginkgo biloba, L-arginine 1

Obsolete Treatments

Chemical or surgical sympathectomy are obsolete and without long-term positive effects. 6 Upper extremity sympathectomy has not been recommended for nonhealing finger ulcers in over 20 years. 5