Raynaud's Phenomenon: Evaluation and Management
Initial Evaluation
Distinguish primary from secondary Raynaud's immediately, as this determines treatment intensity and prognosis. 1
Key Clinical Features to Assess
- Primary Raynaud's presents with symmetric digital attacks, absence of digital ulcers or tissue necrosis, no systemic symptoms, and normal peripheral pulses 2
- Secondary Raynaud's red flags include severe painful episodes, digital ulceration or gangrene (occurs in 22.5% of systemic sclerosis patients), asymmetric attacks or whole-hand involvement, and associated systemic symptoms (joint pain, skin thickening, weight loss, fever) 1, 2
- Systemic sclerosis is the most common underlying disease in secondary Raynaud's 1, 3
Essential Laboratory Workup
When secondary Raynaud's is suspected, order:
- Complete blood count with differential, ESR, ANA, rheumatoid factor, anticentromere and anti-Scl-70 antibodies 1
- Anticardiolipin antibodies and lupus anticoagulant if prothrombotic state suspected 1
- Ankle-brachial index only if peripheral arterial disease is suspected, not routinely 2
Non-Pharmacological Management (All Patients)
Trigger avoidance and lifestyle modifications are mandatory first steps before considering medications. 1
- Absolute smoking cessation 2
- Wear mittens (not gloves), insulated footwear, coat, and hat in cold conditions 1
- Discontinue offending medications: beta-blockers, ergot alkaloids, clonidine, bleomycin 1, 2
- Physical therapy to stimulate blood flow and heat generation 1
First-Line Pharmacological Therapy
Dihydropyridine calcium channel blockers, specifically nifedipine 30-90 mg daily, are the first-line medication for Raynaud's requiring pharmacological treatment. 1, 2
- Nifedipine reduces both frequency and severity of attacks in approximately two-thirds of patients 1
- Meta-analyses of randomized controlled trials confirm efficacy 1
- Other dihydropyridine calcium channel blockers can be substituted if nifedipine is poorly tolerated 1
- Common adverse effects include hypotension, peripheral edema, and headaches 4
Second-Line Pharmacological Therapy
When calcium channel blockers provide inadequate response, add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil). 1, 2
- PDE5 inhibitors effectively reduce frequency, duration, and severity of attacks 1
- Particularly valuable when digital ulcers are present, as they promote both healing and prevention 1
- Cost and off-label use may limit utilization 1
Third-Line Therapy for Severe Refractory Cases
Intravenous prostacyclin analogues (iloprost) should be used for severe Raynaud's unresponsive to oral therapies. 1, 2
- Iloprost is the only prostacyclin analogue proven effective for systemic sclerosis-associated Raynaud's 1
- Particularly effective for healing existing digital ulcers 1
- Reserved for severe digital ischemia with ulceration or gangrene 5
Digital Ulcer Management
For prevention of new digital ulcers in systemic sclerosis, bosentan (endothelin receptor antagonist) is the evidence-based choice, especially with ≥4 baseline ulcers. 1
Prevention Strategy
- Bosentan prevents new digital ulcers but does not improve healing of existing ulcers 1
- PDE5 inhibitors are effective for both prevention and healing 1
Healing Strategy
- Intravenous iloprost has proven efficacy for healing existing digital ulcers 1
- PDE5 inhibitors improve healing rates 1
- Wound care by specialized nurses/physicians, antibiotics only when infection suspected, and adequate pain control are essential 1
Treatment Algorithm by Severity
Mild Raynaud's
- Non-pharmacological measures alone 1
- Add nifedipine if symptoms significantly affect quality of life 1
Moderate Raynaud's or Inadequate Response to CCBs
Severe Raynaud's with Frequent Attacks Despite Above
Digital Ulcers Present
- Bosentan for prevention (especially if multiple ulcers) 1
- Iloprost or PDE5 inhibitors for healing 1
- Immediate reassessment required 2
Common Pitfalls to Avoid
- Do not delay workup for secondary causes when red flags are present—digital ulcers and gangrene indicate urgent need for aggressive therapy 2
- Do not use beta-blockers in patients with Raynaud's, as they can induce or worsen symptoms 1, 3
- Do not assume primary Raynaud's in patients with asymmetric attacks, severe pain, or systemic symptoms—these require full autoimmune workup 2
- Do not use bosentan for healing existing ulcers—it only prevents new ones 1