What is the treatment for Raynaud's disease?

Treatment of Raynaud's Phenomenon

All patients with Raynaud's should implement trigger avoidance and lifestyle modifications first, followed by nifedipine as first-line pharmacotherapy, with escalation to phosphodiesterase-5 inhibitors or intravenous prostacyclin analogues for inadequate response or severe disease. 1

Non-Pharmacological Management (Mandatory First Step)

Trigger avoidance and lifestyle modifications must be implemented before or alongside any pharmacotherapy. 1

• Cold avoidance through proper warm clothing (coat, mittens, hat, insulated footwear, hand/foot warmers) reduces frequency and severity of attacks 2, 1
• Smoking cessation is mandatory as tobacco directly worsens vasospasm and must be addressed 1
• Discontinue triggering medications including beta-blockers, ergot alkaloids, bleomycin, and clonidine 2, 1
• Stress management techniques help reduce attack frequency since emotional stress triggers vasospasm 1
• Avoid vibration injury and repetitive hand trauma, particularly in occupational settings 1
• Physical therapy with exercises to generate heat and stimulate blood flow provides benefit 2, 1

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. 1

• Reduces both frequency and severity of attacks with acceptable adverse effects and low cost 1
• Meta-analyses of randomized controlled trials confirm efficacy 1
• Other dihydropyridine calcium channel blockers can be substituted if nifedipine is poorly tolerated 1
• Common adverse effects include hypotension, peripheral edema, and headaches 3

Second-Line: Phosphodiesterase-5 Inhibitors

For inadequate response to calcium channel blockers, add or switch to PDE5 inhibitors (sildenafil or tadalafil). 1

• Effectively reduce frequency and severity of Raynaud's attacks 1
• Also effective for both healing and prevention of digital ulcers, though prevention data are mixed 2, 1
• Cost and off-label use may limit utilization 2

Third-Line: Intravenous Prostacyclin Analogues

For severe Raynaud's unresponsive to oral therapies, use intravenous iloprost (prostacyclin analogue). 1

• Proven efficacy for reducing frequency and severity of attacks 1
• Superior for healing digital ulcers compared to oral agents 2, 1
• Disadvantaged by parenteral route of administration 4

Management of Digital Ulcers (Secondary Raynaud's)

Prevention of New Digital Ulcers

Bosentan (endothelin receptor antagonist) prevents new digital ulcers, especially in patients with ≥4 existing ulcers. 2, 1

• Does not improve healing of existing ulcers 2
• Most effective in systemic sclerosis patients with multiple digital ulcers 2

Healing of Existing Digital Ulcers

Use PDE5 inhibitors or intravenous iloprost for healing digital ulcers. 2, 1

• Intravenous iloprost has proven efficacy for healing 2, 1
• PDE5 inhibitors improve healing rates 2, 1
• Specialized wound care by trained nurses and physicians may be needed 2
• Add antibiotics only when infection is suspected 2
• Control pain aggressively 2

Severe Complications

In systemic sclerosis, gangrene occurs in 22.5% and osteomyelitis in 11% of patients with digital ulcers. 2

• Amputation may be required for gangrene or osteomyelitis 2

Ancillary and Emerging Therapies

• Topical nitroglycerin can be used as adjunctive therapy 2
• Atorvastatin showed potential for preventing new digital ulcers in small trials but is not included in major guidelines 2
• Digital sympathectomy has evidence for both healing and prevention of digital ulcers 2
• Botulinum toxin infiltrations show promise for healing and prevention in small trials 2
• Fat grafting has supportive evidence for healing digital ulcers 2

Critical Pitfalls to Avoid

Always evaluate for systemic sclerosis and other connective tissue diseases, as delayed diagnosis leads to digital ulcers and poor outcomes. 1

• Secondary Raynaud's requires more aggressive therapy than primary disease 1
• Continuing triggering medications (beta-blockers, vasoconstrictors) will undermine all treatment efforts 1
• Delaying escalation in secondary Raynaud's leads to digital ulcers and poor outcomes 1
• Red flags for secondary disease include severe painful episodes, digital ulceration, and associated systemic symptoms 5, 6

Treatment Intensity Based on Disease Type

Primary Raynaud's typically responds to lifestyle modifications and calcium channel blockers if symptoms affect quality of life 1, 7

Secondary Raynaud's (especially systemic sclerosis) requires earlier and more aggressive pharmacological intervention with readiness to escalate to PDE5 inhibitors or prostacyclin analogues 2, 1