What are the diagnostic criteria and treatment options for Raynaud's disease?

Raynaud's Phenomenon: Diagnosis and Treatment

Diagnostic Approach

Raynaud's phenomenon is diagnosed clinically based on episodic vasospasm of digital arteries causing color changes (pallor, cyanosis, rubor) triggered by cold exposure or emotional stress, with the critical distinction being between primary (idiopathic) and secondary forms. 1, 2

Clinical Criteria for Diagnosis

Primary Raynaud's:

• Isolated vasospastic attacks without underlying disease 1
• Typically affects individual digits rather than entire hand 2
• Mild symptoms without tissue damage 3
• Younger age at onset (often affects younger women) 4, 5
• No systemic symptoms 2

Secondary Raynaud's:

• Associated with underlying connective tissue disease, most commonly systemic sclerosis 1, 2
• May involve entire hand rather than just individual digits 2, 6
• Severe, painful episodes with potential for digital ulcers, gangrene, or osteomyelitis 1, 2
• Older age at onset 3
• Presence of systemic symptoms: joint pain, skin changes, dysphagia, weight loss, malaise, fatigue, fever, photosensitivity, dry eyes, dry mouth 2

Key Physical Examination Findings

Look specifically for:

• Hands: Joint deformities (rheumatoid arthritis), splinter hemorrhages under nails 7
• Skin: Scleroderma changes, facial rosacea, seborrhea 7
• Digital examination: Ulcers, tissue necrosis, gangrene 1, 2
• Nailfold capillaroscopy findings: Abnormal patterns suggest secondary Raynaud's 8

Laboratory Evaluation

For suspected secondary Raynaud's, obtain:

• Antinuclear antibody (ANA) testing 8
• Erythrocyte sedimentation rate (ESR) 8
• Anti-Sjögren syndrome A antibody if dry eyes/mouth present 7
• Viral serology (HBV, HCV, HIV) if systemic disease suspected 7
• Prothrombotic workup if vascular thrombosis suspected: protein C, protein S, antithrombin III, Factor V Leiden, prothrombin mutations, lupus anticoagulant, anticardiolipin antibody 1, 2

Red Flags Requiring Immediate Evaluation

These findings mandate aggressive workup for secondary causes:

• Severe, painful episodes with digital ulceration 1, 2
• Tissue necrosis or gangrene 2
• Fever (rare in primary Raynaud's) 2
• Associated systemic symptoms 2
• Entire hand involvement 2, 6

Treatment Algorithm

Non-Pharmacological Management (All Patients)

First-line for all patients regardless of primary or secondary classification:

• Cold avoidance: Wear coat, mittens (not gloves), hat, insulated footwear 1
• Smoking cessation: Mandatory 1, 3
• Avoid triggers: Trauma, stress, vibration injury, direct contact with cold surfaces 1, 6
• Physical therapy: Exercises to stimulate blood flow and generate heat 1, 6
• Thorough drying: After moisture exposure 6

Pharmacological Treatment for Primary Raynaud's

Mild symptoms affecting quality of life:

• Nifedipine (dihydropyridine calcium channel blocker): First-line pharmacotherapy due to clinical benefit, low cost, acceptable adverse effects 7, 1
• Alternative dihydropyridine CCBs if nifedipine not tolerated 7, 1

Moderate symptoms with inadequate response to CCBs:

• Add or switch to phosphodiesterase-5 inhibitors (sildenafil or tadalafil): Reduces frequency and severity of attacks 7, 1

Severe symptoms despite above treatments:

• Intravenous prostacyclin analogues (iloprost): For refractory cases 7, 1, 5

Pharmacological Treatment for Secondary Raynaud's

Initial therapy:

• Nifedipine or other dihydropyridine CCB: First-line 7, 1

Inadequate response to CCBs:

• Phosphodiesterase-5 inhibitors (sildenafil, tadalafil): Second-line, effective for both attacks and digital ulcer healing/prevention 7, 1

Severe Raynaud's unresponsive to oral therapies:

• Intravenous iloprost (prostacyclin analogue): Third-line for severe attacks and digital ulcer healing 7, 1

Digital ulcer prevention (especially systemic sclerosis with multiple ulcers):

• Bosentan (endothelin receptor antagonist): Prevents new digital ulcers but does not improve healing 7, 1, 3
• PDE5 inhibitors: Effective for both healing and prevention 7, 1

Critical ischemia or pharmacologic failure:

• Botulinum toxin injection: Consider for refractory cases 9
• Digital sympathectomy: Surgical option for severe cases 9
• Amputation: Required in extreme cases with gangrene or osteomyelitis 1

Additional Pharmacological Options (Limited Evidence)

• Fluoxetine (SSRI): May reduce attack frequency, but evidence limited to small studies 1
• Topical nitrates: Alternative vasodilator, though limited by headache, flushing, hypotension 3, 9

Treatments with Insufficient Evidence

Not recommended as primary therapy:

• Biofeedback, acupuncture, ceramic-impregnated gloves 1
• Antioxidants, essential fatty acids, Ginkgo biloba, L-arginine 1

Common Pitfalls

Avoid these errors:

• Missing secondary causes: Always evaluate for systemic sclerosis in patients with severe symptoms, digital ulcers, or older age at onset 1, 2
• Delaying treatment in secondary Raynaud's: Digital ulcers occur in 22.5% of systemic sclerosis patients; early aggressive therapy prevents complications 2
• Using beta-blockers: These can induce or worsen Raynaud's 1, 2
• Prescribing ergot alkaloids, bleomycin, or clonidine: Known to induce Raynaud's 1, 2
• Ignoring occupational factors: Vibration injury and chronic cold exposure are reversible causes 1, 2

Monitoring and Follow-up

For secondary Raynaud's:

• Regular monitoring for digital ulcers, gangrene, and progression of underlying disease 6
• In systemic sclerosis: 22.5% develop digital ulcers, 11% develop gangrene 2
• Reassess treatment efficacy at 3-6 months and escalate therapy if inadequate response 7