What is the recommended management for Raynaud's phenomenon?

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 impair quality of life, with escalation to phosphodiesterase-5 inhibitors for inadequate response and intravenous iloprost for severe refractory disease. 1, 2

Non-Pharmacological Management (Essential for All Patients)

Implement these measures before or alongside any medication:

• Cold avoidance: Wear mittens (not gloves), insulated footwear, coat, hat, and use hand/foot warmers in cold environments 1, 2
• Mandatory smoking cessation: Tobacco directly worsens vasospasm and undermines all treatment efforts 1, 2
• Discontinue triggering medications: Beta-blockers, ergot alkaloids, bleomycin, and clonidine must be stopped or substituted 1, 2
• Stress management techniques: Emotional stress triggers attacks and requires behavioral interventions 2
• Avoid vibration injury and repetitive hand trauma: Particularly important in occupational settings 2
• Physical therapy: Exercises to generate heat and stimulate blood flow provide additional benefit 1, 2

Pharmacological Treatment Algorithm

First-Line: Calcium Channel Blockers

Nifedipine (dihydropyridine-type) is the gold standard first-line therapy, reducing both frequency and severity of attacks in approximately two-thirds of patients. 1, 2

• Start with extended-release nifedipine to minimize adverse effects (headache, ankle swelling, flushing) 1
• Alternative dihydropyridine calcium channel blockers can be substituted if nifedipine is poorly tolerated 1
• This recommendation applies to both primary and secondary Raynaud's phenomenon 1, 2

Second-Line: Phosphodiesterase-5 Inhibitors

Add or switch to sildenafil or tadalafil when calcium channel blockers provide inadequate symptom control. 1, 2

• These agents effectively reduce frequency, duration, and severity of attacks 1
• Particularly valuable if digital ulcers are present, as they promote both healing and prevention 1, 2
• Cost and off-label use may limit utilization but should not prevent appropriate escalation 1

Third-Line: Intravenous Prostacyclin Analogues

Intravenous iloprost is indicated for severe Raynaud's unresponsive to oral therapies. 1, 2

• Proven efficacy for reducing frequency and severity of attacks 1
• Particularly effective for healing existing digital ulcers 1, 2
• Reserved for refractory cases with severe digital ischemia 1

Management of Digital Ulcers in Secondary Raynaud's

Prevention of New Digital Ulcers

Bosentan (endothelin receptor antagonist) is most effective for preventing new digital ulcers, particularly in systemic sclerosis patients with ≥4 existing ulcers. 1, 2

• Does not improve healing of existing ulcers, only prevents new ones 1
• Phosphodiesterase-5 inhibitors also prevent new digital ulcers and offer dual benefit of healing existing ulcers 1, 2

Healing Existing Digital Ulcers

Use intravenous iloprost or phosphodiesterase-5 inhibitors for active digital ulcer healing. 1, 2

• Wound care by specialized nurses/physicians is essential 1
• Antibiotics only when infection is clinically suspected 1
• Adequate pain control is mandatory 1

Treatment Algorithm Based on Disease Severity

Mild Primary Raynaud's

• Non-pharmacological measures alone may suffice 1, 2
• Add nifedipine only if symptoms significantly affect quality of life 1

Moderate to Severe Raynaud's or Inadequate Response to Nifedipine

• Add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil) 1, 2
• Continue non-pharmacological measures 2

Severe Refractory Raynaud's with Frequent Attacks Despite Above Treatments

• Escalate to intravenous iloprost 1, 2
• Consider digital sympathectomy for persistent cases 1

Secondary Raynaud's with Digital Ulcers

• For prevention: Bosentan (if ≥4 ulcers), phosphodiesterase-5 inhibitors, or prostacyclin analogues 1, 2
• For healing: Intravenous iloprost or phosphodiesterase-5 inhibitors 1, 2

Critical Pitfalls to Avoid

Always evaluate for systemic sclerosis and other connective tissue diseases in any patient presenting with Raynaud's phenomenon. 2

• Delayed diagnosis leads to digital ulcers, gangrene, and poor outcomes 2
• Red flags include: severe painful episodes, digital ulceration, onset after age 30, associated systemic symptoms (joint pain, skin changes, dysphagia) 1, 3
• Order appropriate laboratory tests when secondary Raynaud's is suspected: complete blood count, erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, anticentromere and anti-Scl-70 antibodies, anticardiolipin antibodies, and lupus anticoagulant 1

Never continue triggering medications—this will undermine all treatment efforts. 2

• Beta-blockers and other vasoconstrictors must be discontinued or substituted 2

Do not delay escalation in secondary Raynaud's phenomenon. 2

• Secondary Raynaud's requires more aggressive therapy than primary disease 1
• Digital ulcers occur in 22.5% of systemic sclerosis patients, and gangrene in 11% 3
• Early aggressive treatment prevents irreversible complications 2