Raynaud Phenomenon

📋 Key Information Summary

📋

Raynaud phenomenon (RP) is episodic vasospasm of digital arteries causing colour changes (white → blue → red) triggered by cold or emotional stress; prevalence ~3–5% in Australia.
Primary RP (Raynaud disease) is benign, symmetric, with no tissue necrosis, negative autoantibodies, and normal nailfold capillaries — accounts for ~80–90% of cases.
Secondary RP is associated with connective tissue disease (especially systemic sclerosis), has asymmetric onset, digital pitting/ulcers, abnormal nailfold capillaries, and positive ANA/anti-centromere/anti-Scl-70 antibodies.
Nailfold capillaroscopy is the single most important investigation to differentiate primary from secondary RP — giant capillaries and haemorrhages indicate early systemic sclerosis.
ANA testing is recommended for all RP patients; a positive ANA >1:160 with specific extractable nuclear antigens (anti-centromere, anti-Scl-70, anti-RNA polymerase III) warrants rheumatology referral.
First-line pharmacotherapy for symptomatic RP is a dihydropyridine calcium channel blocker — nifedipine slow-release 20–60 mg daily or amlodipine 5–10 mg daily.
Sildenafil 20 mg TDS or tadalafil 20 mg on alternate days is second-line therapy when CCBs are ineffective or not tolerated; neither is PBS-listed for RP.
Digital ulcers occur in ~30% of systemic sclerosis patients; bosentan 62.5 mg BD (increasing to 125 mg BD) is PBS Authority Required for prevention of new digital ulcers in scleroderma.
Lifestyle measures (smoking cessation, warm clothing, avoiding vasoconstrictive drugs, hand-warmers) are essential in all patients and may be sufficient for mild primary RP.
Aboriginal and Torres Strait Islander peoples may have higher prevalence of autoimmune conditions contributing to secondary RP; remote access to rheumatology and capillaroscopy is limited — telehealth and RFDS referral pathways should be used.
Emergency IV iloprost infusion is indicated for critical digital ischaemia with threatened gangrene — available at major tertiary centres.
Avoid β-blockers, ergotamine, sumatriptan, clonidine, and amphetamines in all RP patients as these worsen vasospasm.

Introduction & Australian Epidemiology

Raynaud phenomenon (RP) is a reversible vasospastic disorder of the digital arteries and cutaneous microcirculation, characterised by episodic colour changes of the digits — typically pallor (white), followed by cyanosis (blue) and reactive hyperaemia (red) — provoked by cold exposure or emotional stress. The condition may be primary (idiopathic, benign) or secondary to an underlying disease, most commonly a connective tissue disorder.

In Australia, primary RP affects approximately 3–5% of the general population, with a higher prevalence in women (female-to-male ratio ~4:1), young adults aged 15–30 years, and those with a family history. Secondary RP is estimated to affect 0.5–1% of the population and is most frequently associated with systemic sclerosis (scleroderma), where it is present in >95% of cases. The Australian Scleroderma Cohort Study has documented that digital ulcers — a serious complication of secondary RP — occur in approximately 30% of systemic sclerosis patients and are associated with significant morbidity including pain, infection, and digital amputation.

Cold ambient temperatures in southern Australian states (Victoria, Tasmania, South Australia) exacerbate symptoms. Primary RP is generally self-limiting and does not progress to tissue injury, whereas secondary RP may lead to digital pitting, ulceration, gangrene, and auto-amputation. Correct classification is therefore critical to guide investigation, treatment, and follow-up.

Primary vs Secondary Raynaud

Distinguishing primary from secondary RP is the cornerstone of management. Primary RP (Raynaud disease) is a benign vasospastic condition without underlying pathology, while secondary RP (Raynaud syndrome/phenomenon) is a manifestation of another disease — most commonly a connective tissue disease.

Feature	Primary RP	Secondary RP
Age of onset	15–30 years	>30 years (variable)
Sex	Female >> Male (4:1)	Depends on underlying disease
Symmetry	Symmetric, all digits	Often asymmetric, may spare thumbs
Tissue necrosis	Never	Digital pitting, ulcers, gangrene
Nailfold capillaries	Normal	Abnormal (giant capillaries, haemorrhages)
ANA	Negative or low-titre	Often positive (>1:160)
Specific autoantibodies	Absent	Anti-centromere, anti-Scl-70, anti-RNA Pol III
ESR / inflammatory markers	Normal	May be elevated
Associated features	None	Skin tightening, arthritis, sicca, dysphagia, ILD

⚠️

Red flags for secondary RP — refer to rheumatology: Age of onset >30 years, asymmetric attacks, digital pitting or ulceration, abnormal nailfold capillaries, positive ANA >1:160, abnormal ESR/CRP, or any clinical features of connective tissue disease (skin tightening, rash, arthritis, sicca symptoms, unexplained dyspnoea).

Risk factors for progression from primary to secondary RP include a positive ANA at baseline, abnormal nailfold capillaroscopy, and older age at onset. Longitudinal studies suggest approximately 10–15% of patients initially diagnosed with primary RP will develop features of a connective tissue disease over 10 years of follow-up.

Nailfold Capillaroscopy

Nailfold capillaroscopy (NFC) is the gold-standard bedside and specialist investigation for differentiating primary from secondary RP. It examines the microvascular architecture at the nailfold — the area where cutaneous capillary loops are visible — using a handheld dermatoscope, ophthalmoscope, or stereomicroscope.

Technique

Examine at least 8 fingers (exclude thumbs which have anatomical variation).
Apply a drop of immersion oil or gel to the nailfold to improve optical contact.
Use ×200 magnification (stereomicroscope) for detailed assessment or a dermatoscope/USB videocapillaroscope for clinic-based screening.
Allow the patient to acclimatise to room temperature for 15–20 minutes before examination.

Normal (Primary RP) Findings

Regular, homogenous capillary loops arranged in parallel rows.
No capillary enlargement, haemorrhages, or dropout.
Normal capillary density: 9–14 loops per mm.

Abnormal (Secondary RP — Scleroderma Pattern) Findings

Early

Giant capillaries

Enlarged capillary loops (>50 µm diameter), isolated microhaemorrhages, relatively preserved capillary density.

Setting: Rheumatology — monitor every 6–12 months

Active

Capillary haemorrhages & dropout

Multiple giant capillaries, frequent haemorrhages, irregular capillary architecture, reduced density, some avascular areas.

Setting: Rheumatology — initiate/enhance immunosuppression

Late

Neoangiogenesis & avascular areas

Extensive avascular zones, bushy/ramified neovascular capillaries, marked capillary loss, architectural disorganisation.

Setting: Rheumatology — aggressive management, digital ulcer prevention

ℹ️

Availability in Australia: NFC is available at most tertiary rheumatology departments and some private rheumatology practices. USB videocapillaroscopes are increasingly used in clinic settings. It is not currently a separate MBS item but is performed as part of a specialist consultation. Telehealth NFC using patient-acquired images is an emerging approach for remote and rural patients.

Vasodilator Therapy (CCBs, Sildenafil)

Pharmacotherapy is indicated when lifestyle measures are insufficient to control RP symptoms, particularly when attacks are frequent, prolonged, or interfere with daily activities and work. All patients with secondary RP and digital ischaemia should receive vasodilator therapy.

First-Line: Dihydropyridine Calcium Channel Blockers

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Nifedipine

Adalat® · Slow-release · Dihydropyridine CCB

Adult dose 20 mg SR PO daily, titrate to 30–60 mg SR PO daily (max 90 mg/day)

Paediatric dose 0.25–0.5 mg/kg/day PO in 2–3 divided doses (immediate release); specialist guidance recommended

Route / Frequency Oral, once daily (SR formulation)

Renal adjustment No dose adjustment required; use with caution in severe impairment

Hepatic adjustment Start at lower dose (10–20 mg SR) in hepatic impairment; titrate cautiously

PBS status ✔ PBS General Benefit

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Amlodipine

Norvasc® · Generic · Dihydropyridine CCB

Adult dose 5 mg PO daily, titrate to 10 mg PO daily

Paediatric dose 0.06–0.3 mg/kg/day PO; max 5 mg/day for 6–17 years

Route / Frequency Oral, once daily

Renal adjustment No dose adjustment required

Hepatic adjustment Start at 2.5 mg; titrate cautiously in hepatic impairment

PBS status ✔ PBS General Benefit

Common adverse effects include peripheral oedema, headache, flushing, and dizziness. These are dose-dependent and more common with nifedipine than amlodipine. Sustained-release formulations reduce the incidence of adverse effects. A trial of at least 4 weeks at optimal dose is recommended before determining efficacy.

Second-Line: PDE5 Inhibitors

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Sildenafil

Viagra® · Generic · Phosphodiesterase-5 inhibitor

Adult dose 20 mg PO TDS (off-label for RP); not to be combined with CCBs due to hypotension risk

Paediatric dose Not established for RP in paediatric populations; specialist use only

Route / Frequency Oral, three times daily

Renal adjustment Start at 20 mg PO daily in severe renal impairment (eGFR <30 mL/min)

Hepatic adjustment Start at 20 mg PO daily in hepatic impairment (Child-Pugh B/C)

PBS status ✘ Not PBS listed for RP

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Tadalafil

Cialis® · Generic · Phosphodiesterase-5 inhibitor

Adult dose 20 mg PO on alternate days or 20 mg PO daily (off-label for RP)

Route / Frequency Oral, alternate days or daily

Renal adjustment Max 20 mg on alternate days if eGFR 30–50; avoid if eGFR <30 (dialysis patients: limited data)

Hepatic adjustment Avoid in severe hepatic impairment (Child-Pugh C)

PBS status ✘ Not PBS listed for RP

⚠️

Contraindications for PDE5 inhibitors: Concurrent use with nitrates (risk of severe hypotension), severe cardiovascular instability, recent stroke or MI (within 6 months), severe hepatic impairment, and hereditary retinal disorders. Do not combine PDE5 inhibitors with CCBs without careful blood pressure monitoring.

Other Vasodilator Options (Less Commonly Used)

Agent	Class	Dose	PBS
Losartan	ARB	25–50 mg PO daily	General Benefit
Fluoxetine	SSRI	20 mg PO daily	General Benefit
Atorvastatin	Statin (pleiotropic)	40 mg PO daily (investigational)	General Benefit
Iloprost (IV)	Prostacyclin analogue	0.5–2 ng/kg/min IV infusion over 6 hours × 3–5 days	Authority Required

IV iloprost is reserved for critical digital ischaemia with threatened gangrene and is available at major tertiary rheumatology centres in Australia. Continuous cardiac monitoring is required during infusion due to risk of hypotension and tachycardia.

Digital Ulcers & Bosentan

Digital ulcers are a major complication of secondary RP, occurring in approximately 30% of patients with systemic sclerosis. They result from intimal proliferation, thrombosis, and ischaemia of digital arteries, often complicated by infection. Digital ulcers cause severe pain, functional impairment, and may lead to osteomyelitis, gangrene, and auto-amputation.

Classification of Digital Lesions

Type	Description	Management
Digital pitting	Small depressions at fingertips from healed ulcers	Vasodilators, skin care
Ischaemic digital ulcer	Punched-out lesion with pale or necrotic base on finger pad or periungual area	Vasodilators, wound care, consider bosentan/IV iloprost
Calcific ulcer	Ulcer overlying calcinosis deposits	Surgical debridement, CO₂ laser, topical sodium thiosulfate
Gangrene	Tissue necrosis — dry or wet	IV iloprost, antibiotics, surgical review

Bosentan for Digital Ulcer Prevention

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Bosentan

Tracleer® · Generic · Endothelin receptor antagonist

Adult dose 62.5 mg PO BD for 4 weeks, then increase to 125 mg PO BD

Indication Prevention of new digital ulcers in systemic sclerosis (not treatment of existing ulcers)

Route / Frequency Oral, twice daily, with or without food

Key monitoring LFTs monthly (risk of hepatotoxicity — dose-dependent ALT elevation in ~10%); pregnancy test before initiation (teratogenic — Category X)

Renal adjustment No dose adjustment required; limited data in severe renal impairment

Hepatic adjustment Contraindicated if baseline ALT/AST >3× ULN; reduce dose or discontinue if LFTs rise >5× ULN

PBS status ⚠ PBS Authority Required — for prevention of digital ulcers in systemic sclerosis

🚨

Bosentan is teratogenic — Category X. Pregnancy must be excluded before initiation and reliable contraception (two forms) must be used during treatment and for one month after cessation. Monthly LFT monitoring is mandatory. Bosentan is a potent CYP3A4 and CYP2C9 inducer — check for drug interactions with oral contraceptives, warfarin, simvastatin, and ciclosporin.

Wound Care for Digital Ulcers

Maintain moisture balance — hydrocolloid or foam dressings for clean ulcers.
Debride necrotic tissue; consider surgical debridement for calcinosis-related ulcers.
Swab for culture and sensitivity if signs of infection (erythema, purulence, warmth).
Empirical antibiotics for infected digital ulcers: flucloxacillin 500 mg PO QDS (or cephalexin 500 mg PO QDS if penicillin allergy) — cover S. aureus.
If MRSA suspected or confirmed: doxycycline 100 mg PO BD or TMP-SMX DS PO BD.
Refer to hand surgery/plastic surgery for threatened gangrene or non-healing ulcers.

ANA & Autoantibody Work-Up

Antinuclear antibody (ANA) testing is recommended for all patients presenting with RP to screen for underlying connective tissue disease. The pattern and titre of ANA, combined with extractable nuclear antigen (ENA) profiling, help risk-stratify patients and guide rheumatology referral.

Recommended Work-Up

Essential

ANA by indirect immunofluorescence (IIF) on HEp-2 cells

Negative or low-titre ANA (<1:160) is reassuring. High-titre ANA (≥1:160) warrants further testing. Titre and pattern are both important.

Available

Extractable Nuclear Antigen (ENA) panel

Anti-centromere (ACA), anti-Scl-70 (anti-topoisomerase I), anti-RNA polymerase III, anti-Ro/La, anti-U1 RNP, anti-Smith. Available at major pathology labs (Sullivan Nicolaides, Melbourne Pathology, Douglass Hanly Moir).

Available

Full blood count, ESR, CRP

Screen for anaemia, thrombocytopaenia, and systemic inflammation.

Available

Urea, electrolytes, creatinine, eGFR, LFTs

Baseline organ function; LFTs mandatory before bosentan.

Available

Thyroid function tests

Hypothyroidism is a recognised cause of secondary RP.

Available

Serum protein electrophoresis (SPEP)

Screen for cryoglobulinaemia and paraproteinaemia.

Available

Cryoglobulins

Sample must be kept warm at 37°C during transport. Positive in cryoglobulinaemic vasculitis.

Autoantibody–Disease Associations

Antibody	Associated Condition	Clinical Significance
Anti-centromere (ACA)	Limited cutaneous SSc (CREST)	Higher risk of pulmonary arterial hypertension (PAH); generally slower progression
Anti-Scl-70 (Topo I)	Diffuse cutaneous SSc	Higher risk of interstitial lung disease (ILD); worse prognosis
Anti-RNA Pol III	Diffuse cutaneous SSc	Rapid skin thickening; increased risk of scleroderma renal crisis
Anti-U1 RNP	Mixed connective tissue disease	Overlap features of SSc, SLE, polymyositis
Anti-Ro (SSA) / Anti-La (SSB)	Sjögren syndrome, SLE	Sicca symptoms; neonatal lupus if anti-Ro positive in pregnancy
Anti-Smith, anti-dsDNA	Systemic lupus erythematosus	Lupus-specific; dsDNA correlates with nephritis activity

⚠️

When to refer to rheumatology: ANA ≥1:160, any specific ENA antibody positivity, abnormal nailfold capillaroscopy, digital ulceration/pitting, or clinical features suggestive of connective tissue disease. Early rheumatology involvement improves outcomes in systemic sclerosis through screening echocardiography, pulmonary function testing, and HRCT for ILD.

Lifestyle Measures

Lifestyle modifications are the cornerstone of RP management for all patients — both primary and secondary. These measures reduce attack frequency and severity and may be sufficient alone for mild primary RP.

1

Cold Avoidance

Wear insulated gloves (outer waterproof + inner thermal layers), warm socks, and layered clothing. Use hand-warmers (chemical or rechargeable). Pre-warm car interiors and use heated gloves when outdoors. Avoid reaching into freezers without gloves.

2

Smoking Cessation

Smoking worsens vasospasm and impairs digital blood flow. All RP patients should be strongly encouraged to cease smoking. Offer nicotine replacement therapy, varenicline, or bupropion through PBS-supported programmes. Brief intervention using the 5As framework at every visit.

3

Avoid Vasoconstrictive Medications

Review and cease (where possible): β-blockers (propranolol, atenolol), ergotamine, sumatriptan, clonidine, amphetamines, pseudoephedrine, methylphenidate, ciclosporin. Switch β-blockers to cardioselective agents (bisoprolol) or alternative antihypertensives if cardiac indication exists.

4

Stress Management

Emotional stress is a recognised trigger for RP attacks. Cognitive behavioural therapy, biofeedback, and stress-reduction techniques may be helpful. Referral to psychology for patients with significant anxiety-related RP triggers.

5

Exercise & Skin Care

Regular aerobic exercise promotes peripheral vasodilation. Keep hands and feet well moisturised to prevent skin cracking. Protect digits from trauma — avoid tight jewellery, wear steel-capped boots in occupational settings. Gentle finger exercises during attacks may promote blood flow.

6

Diet & Supplements

Avoid excessive caffeine (vasoconstrictor). Fish oil (omega-3 fatty acids) has modest evidence for reducing RP attack frequency. Ginkgo biloba has limited evidence — not recommended routinely. Ensure adequate vitamin D (common deficiency in SSc patients).

Special Populations

🤰 Pregnancy

Nifedipine Category C — generally considered safe; widely used in pregnancy for hypertension and RP. First-line in pregnancy.

Amlodipine Category C — limited safety data; use nifedipine preferentially.

Sildenafil Category B1 — limited data in pregnancy for RP indication; avoid unless specialist recommendation.

Bosentan Category X — absolutely contraindicated in pregnancy. Ensure contraception before and during treatment.

👶 Paediatrics

Primary RP Most common cause in children. Lifestyle measures first. CCBs (nifedipine or amlodipine at paediatric doses) are first-line if pharmacotherapy needed. Seek paediatric rheumatology input.

Secondary RP Consider juvenile scleroderma, SLE, dermatomyositis. ANA and NFC should be performed. Paediatric rheumatology referral essential.

👴 Elderly

CCBs Start at lower doses (amlodipine 2.5 mg); monitor for hypotension, dizziness, peripheral oedema. Increased fall risk — assess mobility.

Drug interactions Polypharmacy review essential — avoid combining CCBs with other antihypertensives without monitoring. New-onset RP in elderly should prompt consideration of atherosclerotic or thromboembolic cause.

🩺 Renal Impairment

Nifedipine No dose adjustment required. Avoid in scleroderma renal crisis if possible.

Sildenafil Start at 20 mg daily if eGFR <30 mL/min.

🫁 Hepatic Impairment

Bosentan Contraindicated if baseline ALT/AST >3× ULN. Dose-dependent hepatotoxicity — monthly LFTs essential.

CCBs Start at lower doses; amlodipine preferred over nifedipine for hepatic impairment.

🛡️ Immunocompromised

Digital ulcer risk Higher infection risk with digital ulcers in patients on immunosuppression (methotrexate, mycophenolate, rituximab). Lower threshold for antibiotic therapy and surgical review of infected ulcers.

Aboriginal and Torres Strait Islander Health Considerations

Aboriginal and Torres Strait Islander Health

Disease burden

There is limited published data on RP prevalence specifically in Aboriginal and Torres Strait Islander populations. However, autoimmune conditions including systemic sclerosis and SLE may be more prevalent and present at a younger age. Remote communities may be exposed to significant cold stress (Central Australia, Top End wet season) which can exacerbate RP symptoms.

Remote access

Specialist rheumatology services and nailfold capillaroscopy are largely unavailable in remote and very remote communities. Telehealth rheumatology consultations via the RFDS, Aboriginal Community Controlled Health Organisations (ACCHOs), and state-funded telehealth networks are essential pathways. USB videocapillaroscopy via telehealth is an emerging solution.

Medication access

PBS-listed medications (nifedipine, amlodipine) are available through Remote Area Aboriginal Health Services (RAAHS) Section 100 supply. Ensure adequate cold-chain storage for medications. Varenicline or NRT for smoking cessation should be proactively offered. Compounding pharmacies may be required for some vasodilator formulations in very remote areas.

Cultural considerations

Use culturally safe communication with Aboriginal Health Workers and Elders. Explain RP in accessible language — use of visual aids and locally relevant analogies (e.g., "your fingers are like pipes that squeeze shut in the cold"). Respect gender-specific health preferences — some patients may prefer a clinician of the same gender for hand examination. Integrate RP management into existing chronic disease care plans.

Screening & referral

Maintain a low threshold for ANA testing and rheumatology referral in Aboriginal and Torres Strait Islander patients with RP, particularly if digital pitting, ulceration, or skin changes are present. Arrange follow-up through local ACCHOs and ensure continuity of care between remote GP, visiting specialists, and tertiary centres.

Smoking & comorbidity

Smoking rates remain significantly higher in Aboriginal and Torres Strait Islander populations (~40% vs ~11% general population). Smoking cessation is the single most impactful modifiable factor for RP. Integrated care with Tackling Indigenous Smoking programmes and local quit support services should be offered. Comorbid diabetes and peripheral vascular disease may compound digital ischaemia.

📚 References

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