What is the treatment for Raynaud's disease?

Treatment of Raynaud's Phenomenon

Start all patients with trigger avoidance and lifestyle modifications, then add nifedipine as first-line pharmacotherapy if symptoms affect quality of life, escalating to phosphodiesterase-5 inhibitors for inadequate response, and reserving intravenous iloprost for severe refractory disease. 1

Non-Pharmacological Management (Mandatory First Step)

All patients must implement these measures before or alongside any medication 1:

• Cold avoidance: Wear proper warm clothing including coat, mittens (not gloves), hat, insulated footwear, and use hand/foot warmers 1
• Smoking cessation is mandatory: Smoking directly worsens vasospasm and will undermine all treatment efforts 1
• Stop triggering medications: Discontinue beta-blockers, ergot alkaloids, bleomycin, and clonidine 1
• Stress management: Emotional stress triggers attacks and must be addressed 1
• Avoid vibration injury and repetitive hand trauma: Particularly important in occupational settings 1
• Physical therapy: Exercises that generate heat and stimulate blood flow 1

Pharmacological Treatment Algorithm

First-Line: Calcium Channel Blockers

Nifedipine (dihydropyridine-type) is the first-line drug for both primary and secondary Raynaud's 1:

• Reduces both frequency and severity of attacks with acceptable adverse effects and low cost 1
• Meta-analyses of randomized controlled trials confirm efficacy 2
• Common adverse effects include hypotension, peripheral edema, and headaches, which may be reduced by using extended-release preparations 3
• If nifedipine is not tolerated, consider other dihydropyridine calcium channel blockers 2

Second-Line: Phosphodiesterase-5 Inhibitors

Add or switch to sildenafil or tadalafil for inadequate response to calcium channel blockers 1:

• Effectively reduce frequency and severity of attacks 1
• Also effective for both healing and prevention of digital ulcers 1, 2
• Cost and off-label use may limit utilization 2

Third-Line: Intravenous Prostacyclin Analogues

Intravenous iloprost for severe Raynaud's unresponsive to oral therapies 1:

• Proven efficacy for reducing attack frequency and severity 1
• Most effective for healing digital ulcers 1, 4
• Disadvantaged by parenteral route of administration 5

Management of Digital Ulcers

Digital ulcers represent a critical complication requiring aggressive treatment 1:

Prevention of New Digital Ulcers

• Bosentan (endothelin receptor antagonist): Recommended for preventing new digital ulcers, particularly in patients with multiple existing ulcers 1
• Phosphodiesterase-5 inhibitors: Also prevent new digital ulcers, though results are mixed 2

Healing Existing Digital Ulcers

• Intravenous iloprost: Proven most effective for healing 1
• Phosphodiesterase-5 inhibitors: Improve healing of digital ulcers 2
• Local wound care: Soap-and-water washes with either damp dressing or Silvadene cream 6
• Antibiotics as needed for infected ulcers 6
• Pentoxifylline may be added to nifedipine for ulcer management 6

Treatment Algorithm Based on Severity

Mild Primary Raynaud's

• Non-pharmacological measures alone 1
• Add nifedipine if symptoms affect quality of life 2
• Simple vasodilators like naftidrofuryl or inositol nicotinate may be useful with fewer adverse effects 5

Moderate to Severe or Secondary Raynaud's

• Non-pharmacological measures plus nifedipine 1
• Add or switch to phosphodiesterase-5 inhibitors for inadequate response 1
• Consider intravenous iloprost for frequent attacks despite above treatments 2

Severe with Digital Ulcers

• Aggressive pharmacotherapy with bosentan for prevention 1
• Intravenous iloprost or phosphodiesterase-5 inhibitors for healing 1, 2
• In extreme cases with gangrene or osteomyelitis, amputation may be required 2

Critical Pitfalls to Avoid

Always evaluate for systemic sclerosis and other connective tissue diseases 1:

• Delayed diagnosis leads to digital ulcers and poor outcomes 1
• Secondary Raynaud's requires more aggressive therapy than primary 1
• Red flags include severe painful episodes, digital ulceration, and associated systemic symptoms 2, 7

Continuing triggering medications will undermine all treatment efforts 1:

• Beta-blockers and other vasoconstrictors must be discontinued 1
• Review all medications for potential vasospastic effects 1

Do not delay escalation in secondary Raynaud's 1:

• More aggressive therapy is required to prevent digital ulcers and poor outcomes 1
• Digital ulcers occur in 22.5% of systemic sclerosis patients, gangrene in 11% 7

Additional Treatment Considerations

• Fluoxetine (selective serotonin reuptake inhibitor) may be considered, though evidence is limited to small studies 2
• Biofeedback, acupuncture, and ceramic-impregnated gloves have limited evidence 2
• Antioxidants, essential fatty acids, Ginkgo biloba, and L-arginine have minimal evidence 2, 5
• Chemical or surgical sympathectomy is obsolete without long-term positive effects 4
• Fingertip amputation may be necessary for chronic non-healing ulcers with intractable pain, though healing is slow 6