Initial Management of Raynaud's Phenomenon
All patients presenting with Raynaud's phenomenon should immediately implement trigger avoidance and lifestyle modifications, with nifedipine as first-line pharmacotherapy if symptoms affect quality of life or fail to respond to non-pharmacological measures alone. 1
Immediate Non-Pharmacological Interventions (Mandatory First Step)
The American College of Rheumatology mandates that trigger avoidance and lifestyle modifications be implemented before or alongside any pharmacotherapy: 1
- Cold avoidance: Wear proper warm clothing including mittens (not gloves), insulated footwear, coat, and hat; use hand/foot warmers to reduce attack frequency and severity 1, 2
- Smoking cessation: This is mandatory and non-negotiable, as smoking directly worsens vasospasm and will undermine all treatment efforts 1
- Medication review: Immediately discontinue or avoid triggering medications including beta-blockers, ergot alkaloids, bleomycin, and clonidine 1
- Stress management: Implement techniques to reduce emotional stress triggers 1
- Occupational modifications: Avoid vibration injury and repetitive hand trauma, particularly in work settings 1
- Physical therapy: Exercises to generate heat and stimulate blood flow 1
Critical Initial Assessment (Rule Out Secondary Causes)
Always evaluate for systemic sclerosis and other connective tissue diseases immediately, as delayed diagnosis leads to digital ulcers and poor outcomes. 1 Look for these red flags suggesting secondary Raynaud's:
- Severe, painful episodes with digital ulceration 2
- Onset at older age (>30 years) 3
- Associated systemic symptoms: joint pain, skin changes, dysphagia, weight loss, malaise, fatigue, fever, photosensitivity, dry eyes, dry mouth 4
- Involvement of entire hand rather than individual digits 4
If secondary Raynaud's is suspected, order: complete blood count with differential, erythrocyte sedimentation rate, antinuclear antibodies (ANA), rheumatoid factor, anticentromere and anti-Scl-70 antibodies, anticardiolipin antibodies, and lupus anticoagulant 2
First-Line Pharmacotherapy
Nifedipine (dihydropyridine-type calcium channel blocker) is the first-line pharmacological treatment recommended by the European League Against Rheumatism for both primary and secondary Raynaud's. 1, 2 This reduces both frequency and severity of attacks with acceptable adverse effects and low cost, benefiting approximately two-thirds of patients. 2
- Start nifedipine when non-pharmacological measures are insufficient or symptoms affect quality of life 2
- Meta-analyses of randomized controlled trials confirm efficacy in reducing attack frequency and severity 2
- Common adverse effects include hypotension, vasodilatation, peripheral edema, and headaches 3
- Other dihydropyridine calcium channel blockers can be substituted if nifedipine is poorly tolerated 2
Escalation for Inadequate Response
If calcium channel blockers provide inadequate response, add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil), which effectively reduce frequency and severity of attacks. 1, 2 These are particularly valuable if digital ulcers are present, as they promote both healing and prevention. 2
Special Considerations for Secondary Raynaud's
Secondary Raynaud's requires more aggressive therapy and earlier escalation to prevent digital ulcers and poor outcomes. 1 Do not delay escalation, as 22.5% of systemic sclerosis patients develop digital ulcers and 11% develop gangrene. 4
Common Pitfalls to Avoid
- Never continue triggering medications (beta-blockers, vasoconstrictors) as this will undermine all treatment efforts 1
- Do not delay evaluation for connective tissue disease, as this leads to digital ulcers and poor outcomes 1
- Do not undertreat secondary Raynaud's by using the same conservative approach as primary Raynaud's 1
- Do not assume primary Raynaud's without proper workup if red flags are present 2, 4