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Raynaud Phenomenon

NICE CKS Raynaud’s phenomenon. Last revised: Oct 2024.

Raynaud Phenomenon

Raynaud phenomenon describes cold- or stress-induced episodic vasospasm of the arteries or arterioles in the extremities.

Causes and Risk Factors

There are 2 types of Raynaud phenomenon:

Type Description / associated factors
Primary Raynaud phenomenon Occurs in the absence of an underlying cause / disease (“idiopathic”)

+ve Family history is seen in ~30% of cases

Secondary Raynaud phenomenon Associated with an underlying cause, such as:

  • Connective tissue diseases
    • Systemic sclerosis (scleroderma) – classic association
      • >90% of patients with systemic sclerosis have secondary Raynaud phenomenon
    • SLE
    • Mixed connective tissue disease
    • Sjögren’s syndrome
    • Rheumatoid arthritis
    • Dermatomyositis / polymyositis
  • Hypothyroidism
  • Hyperviscosity causes
    • Polycythaemia vera
    • Paraproteinaemias
    • Cryoglobulinaemia
  • Vascular diseases
    • Buerger’s disease
    • Arterial embolisation
    • Atherosclerosis
    • Thoracic outlet syndrome
    • Carpal tunnel syndrome
  • Certain drugs
    • Non-selective beta blockers (e.g. propranolol)
    • Ergot derivatives (e.g. bromocriptine)
    • Amphetamines
    • Cocaine
  • Certain occupational exposure
    • Hand-arm vibration syndrome (from handling vibrating tools)
    • Exposure to certain chemicals (e.g. vinyl chloride, silica, solvents)

Clinical Features and Diagnosis

Raynaud phenomenon itself is a clinical diagnosis: [Ref]

Appearance Classic triphasic phase of an attack:

  • Phase 1: sudden vasospasm → blanching (white)
  • Phase 2: deoxygenation → cyanosis (blue)
  • Phase 3: reactive hyperaemia → reperfusion (red)

The initial phase 1 of blanching must be present to diagnose Raynaud phenomenon. However, the remaining phases are not necessarily always present, and not necessary for diagnosis.

Location Raynaud phenomenon classically and primarily affects the fingers and hands

  • The colour change is usually sharply defined and circumferential (i.e. affecting both the front and the back of the fingers)
  • The colour changes tend to start distally at the fingertips and spread down to 1 or more fingers

Less commonly, other extremities may be affected, such as the tip of the nose, ears, tongue, lips, and areolar tissue

Sensory symptoms
  • Transient numbness is often experienced during the phase 1 blanching
  • Followed by paraesthesia during the phase 3 reperfusion
Triggers
  • Exposure to cold temperature
  • Emotional stress
  • Physically carrying objects

Primary vs secondary Raynaud phenomenon: [Ref]

Primary Raynaud phenomenon Secondary Raynaud phenomenon
  • Onset 10-20 y/o
  • Symmetrical, usually affecting both hands
  • Spare the thumbs
  • Reversible episodes
  • No complications like tissue damage
  • No evidence of underlying cause
  • -ve ANA titre
  • Onset >30 y/o
  • Asymmetrical
  • Thumbs may be affected
  • Episodes are intense, painful and may be irreversible without treatment
  • Complications like tissue damage are possible
  • Evidence of underlying cause
  • +ve ANA titre

Work-Up

Standard work-up in a patient who presents with Raynaud’s phenomenon: [Ref]

Physical examination
  • Assess for trophic changes (digital pitting, nail changes, digital ulcers)
  • Assess for signs of underlying autoimmune or connective tissue disorder (e.g. telangiectasia, sclerodactyly, skin tightening around the mouth, malar rash, synovitis, patchy alopecia)
  • Exclude obstructive vascular disease or distal arterial disease
    • Check peripheral pulses
    • Measure BP
    • Perform Allen’s test
  • Capillaroscopy or dermatoscopy of the nail fold
    • Abnormal nailfold microcirculation suggests secondary Raynaud phenomenon
Investigations
  • Basic blood tests (FBC, TFT, CRP, ESR)
  • Serology
    • ANA in all patients
    • If ANA +ve → ENA screen
  • Urinalysis

Complications

Primary Raynaud phenomenon typically causes reversible episodes which do NOT progress to tissue damage

Secondary Raynaud phenomenon, if undiagnosed and untreated, may progress to permanent tissue damage, including

  • Digital ischaemia → digital pitting, ulceration, tissue necrosis, dry gangrene, telescoping digits, loss of digits
  • Secondary infection, possible osteomyelitis, abscess formation

Management

Referral Criteria

Referral Indications
Hospital admission or urgent rheumatology or vascular surgery referral
  • Severe critical digital ischaemia (suggested by persistent skin colour changes and/or severe ischaemic pain)
  • Digital ulceration and/or necrosis
  • Severe tissue infection
  • Proximal large vessel occlusive vascular disease
Referral to rheumatology
  • Suspected secondary Raynaud phenomenon
  • Frequent, severe episodes of Raynaud phenomenon
Referral to paediatric / paediatric rheumatology
  • ≤12 y/o with suspected Raynaud phenomenon
  • 13-17 y/o being considered for drug treatment

Primary Care Management

Initial management: lifestyle measures Temperature-related measures:

  • Avoid sudden temperature changes
  • Avoid cold exposure
  • Keep the whole body warm, including the hands and feet (e.g. using layered clothing, gloves, footwear, head covering)

Other lifestyle measures:

  • Smoking cessation
  • Reduce caffeine consumption
  • Advise on stress management 
  • Avoid triggers like vibration exposure at work
If lifestyle measures are ineffective: pharmacological management
  • 1st line: calcium channel blockers
    • Preferred: slow-release nifedipine
    • Alternative: amlodipine
  • 2nd line: other vasodilators [Ref]
    • PDE-5 inhibitors (e.g. sildenafil, tadalafil)
    • Topical nitrates
    • Fluxetine
    • Losartan

In those with secondary Raynaud phenomenon, also identify and manage any underlying causes or associated conditions

References

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