Management of Raynaud's Phenomenon

First-Line Management: Non-Pharmacological Measures

All patients with Raynaud's phenomenon must implement trigger avoidance and lifestyle modifications before or alongside any pharmacotherapy. 1

Smoking cessation is mandatory as smoking directly worsens vasospasm and undermines all treatment efforts 1
Cold avoidance measures include wearing proper warm clothing (coat, mittens, hat, dry insulated footwear) and using hand/foot warmers 2, 1
Discontinue triggering medications including beta-blockers, ergot alkaloids, bleomycin, and clonidine 2, 1
Implement stress management techniques as emotional stress triggers 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 can be beneficial 2, 1

Nifedipine (dihydropyridine calcium channel blocker) is the first-line pharmacotherapy for both primary and secondary Raynaud's phenomenon. 1

Nifedipine reduces both frequency and severity of attacks with acceptable adverse effects and low cost 1
Extended-release formulations reduce adverse effects like ankle swelling, headache, and flushing 3
Other dihydropyridine calcium channel blockers (e.g., diltiazem) can be considered if nifedipine is poorly tolerated, though efficacy may be reduced 3

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

PDE5 inhibitors effectively reduce frequency and severity of Raynaud's attacks 1
These agents are also effective for both healing and preventing digital ulcers 2, 1
Cost and off-label use may limit utilization 2

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

Iloprost has proven efficacy for healing digital ulcers 2, 1
Prostacyclin analogues are the most promising drugs for secondary Raynaud's disease 4
Parenteral route of administration is a disadvantage 3

For patients with digital ulcers, treatment strategy depends on whether the goal is healing or prevention:

Bosentan (endothelin receptor antagonist) is first-line for preventing new digital ulcers, especially in patients with ≥4 existing ulcers 2
PDE5 inhibitors can also prevent new digital ulcers, though evidence is mixed 2
Intravenous prostacyclin analogues are effective for prevention 2

Digital sympathectomy can be used for healing and prevention of digital ulcers 2
Botulinum toxin infiltrations show promise for healing and prevention 2
Fat grafting has supportive evidence for healing 2
In cases of gangrene (22.5% of SSc patients) or osteomyelitis (11% of SSc patients), amputation may be required 2

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

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

Continuing triggering medications (beta-blockers, vasoconstrictors) will undermine all treatment efforts 1
Delaying escalation in secondary Raynaud's can lead to digital ulcers and poor outcomes—more aggressive therapy is required 1
Secondary Raynaud's requires more aggressive pharmacological therapy than primary Raynaud's 5
Most drugs are effective in less than 50% of treated patients and do not completely abolish attacks, but reduce severity and frequency 4

Mild Raynaud's: Non-pharmacological measures alone; add nifedipine if symptoms affect quality of life 5
Moderate to severe or inadequate response to CCB: Add or switch to PDE5 inhibitors 5
Severe with frequent attacks despite above: Consider intravenous prostacyclin analogues 5
Digital ulcers present: Use bosentan for prevention; iloprost or PDE5 inhibitors for healing 2, 5