Raynaud's phenomenon
Exam Tips
- For OSCE diagnosis, blanching is the key required colour phase; full triphasic change is not mandatory.
- Differentiate primary vs secondary: young onset, symmetric attacks, thumb sparing, normal nailfolds, and negative/low ANA suggest primary disease.
- Red flags for urgent same-day specialist assessment: persistent rest pain, ulceration, infection, black tissue, asymmetry, or late-onset severe attacks.
- Always screen for connective tissue disease because Raynaud's may precede systemic sclerosis by years.
- Image cue: recognise sequence of pallor -> cyanosis -> erythema in exam photos and compare with nailfold capillary abnormalities (See Figure: nailfold capillaroscopy pattern in systemic sclerosis, standard rheumatology atlas).
Definition
Raynaud's phenomenon is an episodic vasospastic disorder of small digital arteries/arterioles causing transient, reversible ischaemia, typically triggered by cold exposure or emotional stress. The hallmark is sharply demarcated digital blanching (often followed by cyanosis and then reactive erythema on rewarming), with primary disease occurring without an underlying cause and secondary disease linked to conditions such as connective tissue disease.
Pathophysiology
The disorder reflects dysregulated peripheral blood-flow control from interacting neural, vascular, and intravascular mechanisms. In primary Raynaud's, there is an exaggerated sympathetic vasoconstrictor response (including alpha-adrenergic hyperreactivity) in thermoregulatory arteriovenous shunts, often without structural vessel disease. In secondary Raynaud's (especially systemic sclerosis), endothelial dysfunction (reduced vasodilators such as nitric oxide/prostacyclin, increased endothelin-1), platelet activation, and progressive structural vasculopathy (intimal proliferation/lumen narrowing) drive more severe, persistent ischaemia and tissue injury.
Risk Factors
- Female sex (primary Raynaud's is much more common in women)
- Family history of Raynaud's
- Cold climate/exposure
- Smoking
- Manual occupations and vibration-tool exposure (hand-arm vibration syndrome)
- Low BMI
- Connective tissue disease (especially systemic sclerosis, mixed connective tissue disease, SLE, Sjogren's, dermatomyositis/polymyositis, rheumatoid arthritis)
- Vasoactive drugs/substances: non-selective beta-blockers, ergot derivatives, bromocriptine, oestrogens, amphetamines, cocaine, cannabis, excess caffeine, interferons, some cytotoxics
- Associated vasospastic disorders (e. g, migraine, variant angina)
Clinical Features
Symptoms
- Episodic, clearly demarcated finger colour change precipitated by cold or stress
- Blanching attacks (required feature for diagnosis), sometimes with sequential blue then red phases
- Digital numbness, tingling, and cold discomfort during attacks
- Throbbing/pain on rewarming
- In secondary disease: more severe pain, asymmetrical attacks, and symptoms persisting between episodes
Signs
- Circumferential pallor of one or more digits, often starting at fingertips and spreading proximally
- Symmetrical hand involvement with thumb sparing suggests primary Raynaud's
- Digital pitting scars, ulcers, or necrosis suggest secondary Raynaud's with ischaemic complications
- Abnormal nailfold capillaries (e. g, dilated/dropout pattern) suggest connective tissue disease
- Features of underlying disease: sclerodactyly/telangiectasia, synovitis, rash, signs of arterial occlusion
Investigations
Management
Lifestyle Modifications
- Keep whole body warm: layered clothing, insulated gloves/socks, hand warmers, avoid rapid temperature changes
- Stop smoking and avoid nicotine/vaping exposure
- Reduce triggers: stress management, avoid vibrating tools where possible, regular hand protection at work
- Avoid precipitating medicines/substances where safe alternatives exist (e. g, non-selective beta-blockers, sympathomimetics, recreational vasoconstrictors, excess caffeine)
- Skin and hand care to prevent fissures/ulcers; urgent review for new digital ulcer, infection, or black discolouration
Pharmacological Treatment
Dihydropyridine calcium-channel blockers (first-line oral vasodilators)
- Nifedipine modified-release 10 mg twice daily initially, titrate according to response/tolerance (commonly up to 20 mg twice daily; specialist practice may use higher doses)
- Amlodipine 5 mg once daily, increase to 10 mg once daily if needed/tolerated (off-label use for Raynaud's)
Use lowest effective dose; common adverse effects include headache, flushing, ankle oedema, dizziness, and hypotension. Use caution in significant hypotension or severe aortic stenosis; review interactions with other antihypertensives.
PDE-5 inhibitor (specialist use for refractory/severe secondary Raynaud's)
- Sildenafil 25 mg three times daily (titrate in specialist care based on response and adverse effects)
Contraindicated with nitrates or nitric oxide donors due to profound hypotension risk; caution with alpha-blockers and cardiovascular instability.
Prostacyclin analogue infusion (critical ischaemia/severe refractory disease, specialist)
- Iloprost intravenous infusion, dose and schedule per specialist protocol
Used for severe secondary Raynaud's/digital ischaemia; monitor for hypotension, headache, flushing, jaw pain, and infusion-related adverse effects.
Endothelin receptor antagonist (ulcer prevention in systemic sclerosis, specialist)
- Bosentan 62.5 mg twice daily for 4 weeks then 125 mg twice daily
For prevention of new digital ulcers in systemic sclerosis (not for acute attack relief). Hepatotoxic and teratogenic risks require liver-function and pregnancy monitoring; major CYP interactions.
Surgical / Interventional
- Digital sympathectomy for refractory severe ischaemia or non-healing digital ulcers (specialist vascular/plastic surgery)
- Debridement or amputation if irreversible necrosis/gangrene develops
- Manage contributory structural lesions (e. g, thoracic outlet decompression) when indicated
Complications
- Digital pitting scars and chronic fingertip pain
- Digital ulceration
- Acute critical digital ischaemia with tissue necrosis or dry gangrene
- Secondary soft-tissue or bone infection (including osteomyelitis)
- Loss of digital tissue/autoamputation in advanced secondary disease
- Reduced quality of life due to frequent painful attacks and functional impairment
Prognosis
Primary Raynaud's is usually benign, often mild, and may remit over time, with low risk of permanent tissue damage. Secondary Raynaud's has a more aggressive course determined by the underlying condition, particularly connective tissue disease; abnormal nailfold capillaries and positive ANA increase risk of progression and ischaemic complications.
Sources & References
🏥BMJ Best Practice(1)
💊BNF Drug References(2)
- Iloprost[management.pharmacological]
- Nifedipine[management.pharmacological]
✅NICE Guidelines(1)
- Raynaud's phenomenon[overview]