How should Raynaud's phenomenon affecting the hands be managed?

Management of Raynaud's Phenomenon Affecting the Hands

All patients with Raynaud's phenomenon should begin with non-pharmacological 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)

Trigger avoidance and lifestyle modifications must be implemented before or alongside any pharmacotherapy. 2

Critical Lifestyle Interventions:

• Cold avoidance: Wear mittens (not gloves), insulated footwear, hats, and coats; use hand/foot warmers 1, 2
• Smoking cessation is mandatory as tobacco directly worsens vasospasm and undermines all treatment efforts 1, 2
• Discontinue triggering medications: Beta-blockers, ergot alkaloids, bleomycin, and clonidine 1, 2
• Stress management techniques to reduce emotionally-triggered attacks 2
• Avoid vibration injury and repetitive hand trauma, particularly in occupational settings 1, 2
• Physical therapy with exercises to stimulate blood flow and generate heat 1, 2

For patients with systemic sclerosis and puffy hands, manual lymphatic drainage (5 weekly sessions) can improve hand function. 3

Pharmacological Treatment Algorithm

First-Line: Calcium Channel Blockers

Nifedipine (dihydropyridine-type calcium channel blocker) is the gold standard first-line therapy for both primary and secondary Raynaud's, reducing frequency and severity of attacks in approximately two-thirds of patients with acceptable adverse effects and low cost. 1, 2, 4

• Use extended-release formulations to minimize adverse effects (ankle swelling, headache, flushing) 4
• Alternative dihydropyridine calcium channel blockers (felodipine, isradipine, diltiazem) can be considered if nifedipine is not tolerated, though efficacy may be reduced 1, 4

Second-Line: Phosphodiesterase-5 Inhibitors

When calcium channel blockers provide inadequate response, add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil). 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 1

Third-Line: Intravenous Prostacyclin Analogues

For severe Raynaud's unresponsive to oral therapies, intravenous iloprost should be considered. 1, 2

• Proven efficacy for reducing frequency and severity of attacks 1
• Particularly effective for healing existing digital ulcers 1, 2
• Disadvantaged by parenteral route of administration 4

Management of Digital Ulcers

Prevention:

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

• Phosphodiesterase-5 inhibitors also prevent new digital ulcers, though results are mixed 1

Healing:

• Intravenous iloprost has proven efficacy for healing existing digital ulcers 1, 2
• Phosphodiesterase-5 inhibitors improve healing of digital ulcers 1
• Wound care by specialized nurses/physicians, antibiotics only when infection is suspected, and pain control are essential 1

Additional Treatment Options

Adjunctive Therapies:

• Topical nitroglycerin can provide ancillary benefit for acute painful episodes 1
• Fluoxetine (selective serotonin reuptake inhibitor) might be considered, though evidence is limited to small studies 1
• Simple vasodilators (naftidrofuryl, inositol nicotinate, pentoxifylline) are useful in mild disease with fewer adverse effects 4

Interventional Approaches for Refractory Cases:

• Digital sympathectomy can be considered for persistent digital ulcer healing/prevention needs 1
• Botulinum toxin infiltrations or fat grafting have emerging evidence for healing and prevention of digital ulcers 1
• Lumbosacral sympathectomy may cure vasospastic attacks of the toes 5

Critical Pitfalls to Avoid

Always evaluate for systemic sclerosis and other connective tissue diseases, as Raynaud's occurs in nearly all individuals with systemic sclerosis and is frequently the first clinical sign. 1, 2

Red Flags Requiring Urgent Evaluation:

• Severe, painful episodes with digital ulceration or tissue necrosis 1, 6
• Associated systemic symptoms (joint pain, skin changes, dysphagia, weight loss, fever) 6
• Involvement of entire hand rather than individual digits 6
• Onset after age 60 (consider atherosclerotic disease) 4

Delayed diagnosis leads to digital ulcers and poor outcomes—secondary Raynaud's requires more aggressive therapy than primary disease. 2 Continuing triggering medications (beta-blockers, vasoconstrictors) will undermine all treatment efforts. 2

Diagnostic Workup for Secondary Causes:

When secondary Raynaud's is suspected, order: complete blood count with differential, erythrocyte sedimentation rate, antinuclear antibodies, rheumatoid factor, anticentromere and anti-Scl-70 antibodies, anticardiolipin antibodies, and lupus anticoagulant. 1

Severity-Based Treatment Approach

Mild Raynaud's: Non-pharmacological measures alone; add nifedipine if symptoms affect quality of life 1

Moderate to Severe Raynaud's or inadequate response to calcium channel blockers: Add or switch to phosphodiesterase-5 inhibitors 1

Severe Raynaud's with frequent attacks despite above treatments: Consider intravenous iloprost 1

Digital ulcers present: Use bosentan for prevention, intravenous iloprost or phosphodiesterase-5 inhibitors for healing 1, 2

Gangrene or osteomyelitis: Amputation might be required in extreme cases 1