What are the treatment options for Raynaud's disease?

Treatment of Raynaud's Phenomenon

All patients with Raynaud's should implement non-pharmacological measures first, with nifedipine as the first-line medication when pharmacotherapy is needed. 1

Non-Pharmacological Management (First-Line for All Patients)

These lifestyle modifications must be implemented before or alongside any medication: 1

• Cold avoidance through proper warm clothing (coat, mittens, hat, insulated footwear) and hand/foot warmers to reduce attack frequency and severity 1
• Mandatory smoking cessation, as tobacco directly worsens vasospasm and undermines all treatment efforts 1
• Discontinue triggering medications including beta-blockers, ergot alkaloids, bleomycin, and clonidine 1
• Stress management techniques to reduce emotionally-triggered attacks 1
• Avoid vibration injury and repetitive hand trauma, particularly in occupational settings 1
• Physical therapy with exercises to generate heat and stimulate blood flow 1

Pharmacological Treatment Algorithm

First-Line Pharmacotherapy

Nifedipine (dihydropyridine calcium channel blocker) is the gold-standard first-line medication for both primary and secondary Raynaud's, reducing frequency and severity of attacks with acceptable adverse effects and low cost. 1 Meta-analyses of randomized controlled trials confirm its efficacy. 2 Extended-release formulations (30 mg at bedtime) reduce side effects like ankle swelling, headache, and flushing. 3, 4

Second-Line Pharmacotherapy

Phosphodiesterase-5 inhibitors (sildenafil or tadalafil) should be added or switched to when calcium channel blockers provide inadequate response, as they effectively reduce both frequency and severity of attacks. 1, 2 These agents also heal and prevent digital ulcers, though cost and off-label use may limit utilization. 2

Third-Line Pharmacotherapy

Intravenous iloprost (prostacyclin analogue) is reserved for severe Raynaud's unresponsive to oral therapies, with proven efficacy for both reducing attacks and healing digital ulcers. 1, 2 This is the most promising drug for secondary Raynaud's disease. 5

Management of Digital Ulcers

Digital ulcers occur in 22.5% of systemic sclerosis patients and require aggressive treatment: 6

• Bosentan (endothelin receptor antagonist) prevents new digital ulcers, particularly in patients with multiple existing ulcers 1, 2
• Phosphodiesterase-5 inhibitors both prevent new ulcers and heal existing ones 1, 2
• Intravenous iloprost is proven effective for healing digital ulcers 1, 2
• Local wound care with soap-and-water washes, damp dressings or Silvadene cream, plus antibiotics as needed 3
• Fingertip amputation may be necessary for chronic non-healing ulcers with intractable pain, though healing is slow 3

Treatment Stratification by Severity

Mild Raynaud's: Non-pharmacological measures alone; add nifedipine only if symptoms affect quality of life 2

Moderate to severe Raynaud's or inadequate response to nifedipine: Add or switch to phosphodiesterase-5 inhibitors 2

Severe Raynaud's with frequent attacks despite above treatments: Consider intravenous prostacyclin analogues 2

Digital ulcers present: Use bosentan for prevention, iloprost or PDE5 inhibitors for healing 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
• Never continue triggering medications (beta-blockers, vasoconstrictors), as they will undermine all treatment efforts 1
• Do not delay escalation in secondary Raynaud's, as more aggressive therapy is required to prevent digital ulcers 1
• Recognize red flags for secondary Raynaud's: severe painful episodes, digital ulceration/necrosis, associated systemic symptoms (joint pain, skin changes, dysphagia), or onset after age 30 6, 2

Alternative Therapies with Limited Evidence

Fluoxetine (SSRI) may be considered for attacks, though evidence is limited to small studies. 2 Biofeedback, acupuncture, ceramic-impregnated gloves, antioxidants, essential fatty acids, Ginkgo biloba, and L-arginine have minimal supporting evidence. 2, 3

Upper extremity sympathectomy is obsolete and provides no long-term benefit. 3, 5