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Thromboangiitis Obliterans

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Overview

Thromboangiitis obliterans, also known as Buerger’s disease is an inflammatory vasculitis leading to thrombosis of medium- and small-sized vessels. The thrombosis may lead to arterial ischaemia in the distal extremities and superficial thrombophlebitis, possibly leading to gangrene and ulceration.

Smoking tobacco is the strongest risk factor for the development of thromboangiitis obliterans.

Epidemiology

  • Peak incidence is 30-40 years
  • More common in men

Risk Factors & Associations

  • Smoking
  • Age <40 years
  • Male sex

Presentation

  • Ischaemia of the extremities:
    • Cold extremities
    • Changes in skin colour of extremities
    • Pallor of extremities
    • Paraesthesia in extremities
    • Absent or weak distal pulses
    • Positive Allen’s test
  • Claudication
  • Rest pain that can be eased by hanging the legs over the edge of the bed
  • Superficial thrombophlebitis
  • Raynaud’s phenomenon

Differential Diagnoses

Peripheral arterial disease

  • Superficial thrombophlebitis not present
  • Upper extremity involvement is less likely
  • Risk factors for atherosclerotic disease present
  • Claudication is present
  • Absent femoral pulses may be present

Embolic disease

  • Usually affects one limb
  • Recent chest pain suggestive of a myocardial infarction
  • An echocardiogram may show valve lesions or thrombi
  • ECG and troponin may show evidence of myocardial ischaemia

Raynaud’s phenomenon

  • Painful extremities with cold weather
  • All investigations are normal if primary Raynaud’s phenomenon

Systemic lupus erythematosus

  • SLE features are present e.g. malar rush/photosensitivity etc.
  • ANA/anti-dsDNA present 

Investigations

Initially, investigations to rule out other causes of vasculitis should be ruled out:

  • Full blood count (FBC):
    • To screen for myeloproliferative diseases
  • Fasting glucose:
    • To screen for diabetes
  • C-reactive protein(CRP) and erythrocyte sedimentation rate (ESR):
    • Normal but may be elevated if gangrene is present
  • Coagulation assay and thrombophilia screen:
    • To screen for hypercoagulable states
  • Ureaand electrolytes (U&Es):
    • Normal – kidney dysfunction suggests an autoimmune disease
  • Rheumatological autoantibodies – to screen for rheumatological diseases:
    • Anti-nuclear (ANA)
    • Rheumatoid factor
    • Anti-nucleophilic cytoplasmic antibody (ANCA)
    • Anti-centromere antibodies
    • Topoisomerase I antibodies (Scl-70)
  • Echocardiogram:
    • To identify potential embolic sources
  • Arterial duplex:
    • Corkscrew-shaped collateral vessels are present
  • CT angiography/MR angiography:
    • If the arterial duplex is insufficient
    • Corkscrew-shaped collateral vessels are present
  • Biopsy may be considered:
    • This should be avoided in ischaemic tissue
    • This can show inflammatory thrombosis with sparing of the internal elastic lamina

Diagnosis

  • Diagnosis is often made after excluding other vascular diseases
  • The Shinoya criteria are often used to diagnose thromboangiitis obliterans

Management

  • Immediate smoking cessation
  • If patients have critical ischaemia/severe claudication – surgical revascularisation
  • Dry gangrenous limbs should be reviewed monthly to monitor for infection

Patient Advice

  • Patients should be advised to stop smoking

Complications

  • Ulceration
  • Gangrene
  • Amputation secondary to continued smoking
  • Atherosclerosis

Prognosis

  • Thromboangiitis obliterans is progressive in patients who continue to smoke
  • Smoking for >20 years had a significant relationship with major amputations

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