47. Buerger's disease and Raynaud's disease

1. Definition

• Buerger's disease (thromboangiitis obliterans) → non-atherosclerotic, segmental, inflammatory occlusive disease of small and medium arteries, veins and nerves of the limbs, strongly linked to tobacco
• Raynaud's phenomenon → episodic vasospasm of the digital arteries, provoked by cold or emotion, with colour changes of the fingers or toes
  • Raynaud's disease (primary) → no underlying cause
  • Raynaud's syndrome (secondary) → due to another disease (connective tissue disease, drugs, vibration)

2. Epidemiology

Buerger's disease

• Young men, age 20–45 years, heavy smokers; women more often now
• More common in the Middle East, Asia and Eastern Europe

Raynaud's

• Primary → 3–5% of the population; young women (female:male about 9:1); onset age 15–30; often family history
• Secondary → older; systemic sclerosis (> 90% have Raynaud's), SLE

3. Aetiology and risk factors

Buerger's disease

• Tobacco → essential; all forms (cigarettes, cigars, chewing tobacco, cannabis)
• Genetic susceptibility (HLA types)
• Immune reaction against vessel wall collagen

Raynaud's

• Primary → abnormal sympathetic control, raised α2-adrenergic sensitivity, low vasodilators (calcitonin gene-related peptide), high endothelin
• Secondary causes:
  • Connective tissue disease → systemic sclerosis, SLE, rheumatoid arthritis, Sjögren's, dermatomyositis
  • Vibration white finger → vibrating tools
  • Drugs → β-blockers, ergotamine, chemotherapy, cocaine
  • Arterial disease → Buerger's, atherosclerosis, thoracic outlet syndrome
  • Blood → cryoglobulins, cold agglutinins, polycythaemia
  • Hypothyroidism

4. Pathogenesis

Buerger's disease

1. Acute phase → inflammatory thrombus with microabscesses and giant cells; vessel wall structure kept (unlike vasculitis)
2. Subacute phase → thrombus organises, partial recanalisation
3. Chronic phase → fibrosis around artery, vein and nerve together
4. Starts distally (digital, plantar, tibial, radial and ulnar arteries) → spreads upwards
5. Migratory superficial thrombophlebitis in about 40%

Raynaud's phenomenon

1. Cold or stress → digital arteries spasm → white (pallor, ischaemia)
2. Slow flow, deoxygenated blood → blue (cyanosis)
3. Spasm relaxes → reactive hyperaemia → red (rubor), with pain and tingling
4. Secondary → structural damage in the vessels → ulcers, pulp atrophy, gangrene

5. Classification

Buerger's disease: Shionoya criteria

1. Smoking history
2. Onset before age 50
3. Infrapopliteal arterial occlusion
4. Upper limb involvement or superficial migratory phlebitis
5. No atherosclerotic risk factors other than smoking
6. Stages → like PAD (Fontaine): claudication, rest pain, ulcers, gangrene

Raynaud's

• Primary (Raynaud's disease) → symmetrical, no ulcers, normal nailfold capillaries, negative antibodies, normal ESR
• Secondary (Raynaud's syndrome) → asymmetrical, older onset, ulcers, abnormal capillaries, positive antibodies

6. Clinical picture

Symptoms

• Buerger's:
  • Instep (foot) claudication → arch of the foot, typical
  • Rest pain in fingers and toes
  • Cold sensitivity, Raynaud's phenomenon (about 40%)
  • Painful ulcers and gangrene of the digits
  • Painful red cords → migratory thrombophlebitis
• Raynaud's:
  • Attacks of white, then blue, then red fingers on cold exposure
  • Numbness, pain, tingling on rewarming
  • Thumb often spared in primary disease
  • Secondary → digital ulcers, skin thickening, joint pain, dry eyes

Signs

• Buerger's:
  • Absent foot and wrist pulses with normal femoral and popliteal pulses
  • Positive Allen's test → ulnar or radial artery occluded
  • Ulcers, gangrene of fingertips and toes
• Raynaud's:
  • Between attacks often normal
  • Sclerodactyly, calcinosis, telangiectasia (systemic sclerosis)
  • Pitted scars, pulp atrophy

7. Diagnosis

Buerger's disease

• Diagnosis of exclusion → no diabetes, hyperlipidaemia, embolic source or autoimmune disease
• Tests → glucose, lipids, autoantibodies, thrombophilia screen, echocardiography
• Angiography of all four limbs:
  • Segmental occlusion of small and medium distal arteries
  • "Corkscrew" collaterals
  • Normal proximal arteries, no atheroma
• Biopsy → rarely; acute lesion shows inflammatory thrombus

Raynaud's

• Clinical → Allen and Brown criteria for primary disease: attacks by cold or emotion, both hands, no gangrene, no other cause, for at least 2 years
• Nailfold capillaroscopy → dilated, lost capillaries in secondary disease
• Bloods → FBC, ESR, CRP, antinuclear antibodies (ANA), anti-centromere, anti-Scl-70, rheumatoid factor, thyroid function, cryoglobulins
• Digital pressures after cooling, cold provocation test
• Duplex or CT angiography → thoracic outlet, proximal disease

8. Differential diagnosis

• Atherosclerosis (older, risk factors)
• Embolism (heart, thoracic outlet, aneurysm)
• Vasculitis → Takayasu's, polyarteritis nodosa, scleroderma
• Diabetic microangiopathy
• Ergotism
• Acrocyanosis, chilblains (perniosis)
• Thoracic outlet syndrome

9. Treatment

Conservative

• Buerger's:
  • Complete stop of all tobacco → the only treatment proven to halt the disease; amputation risk falls sharply if they stop
  • Nicotine replacement must also stop
  • Foot care, protect from cold and injury
  • Iloprost (prostacyclin) infusion → ulcer healing, rest pain
  • Analgesia, antiplatelet, calcium-channel blockers
  • Treat ulcer infection
• Raynaud's:
  • Keep warm → gloves, heated gloves and socks
  • Stop smoking; avoid β-blockers and vibrating tools
  • Calcium-channel blockers → nifedipine first-line; amlodipine, diltiazem
  • Others → losartan, fluoxetine, sildenafil, topical nitrates
  • Severe or ulcers → IV iloprost; bosentan to prevent new ulcers in systemic sclerosis
  • Treat the underlying disease

Surgical

• Buerger's:
  • Bypass rarely possible (distal disease, no target); poor patency
  • Sympathectomy → lumbar for feet, thoracoscopic for hands; may help ulcer healing and pain
  • Omentoplasty, spinal cord stimulation → selected patients
  • Amputation → digits, minor first; major amputation if they keep smoking
• Raynaud's:
  • Thoracoscopic (T2–T3) sympathectomy → short-term relief; recurrence common
  • Digital (periarterial) sympathectomy, botulinum toxin injection
  • Debridement or digit amputation for gangrene

10. Complications

• Buerger's → digital gangrene, amputation (about 25% of those who keep smoking), chronic pain, disability
• Raynaud's → digital ulcers, infection, pulp loss, gangrene (secondary form)
• Sympathectomy → Horner's syndrome, compensatory sweating, pneumothorax, neuralgia