Makindo Medical Notes

Related Subjects: |Testicular Torsion |Paraphimosis |Meckel's diverticulum |Buerger's disease (Thromboangiitis obliterans )

Thromboangiitis Obliterans (Buerger’s Disease) is a rare, non-atherosclerotic inflammatory vasculitis of unknown etiology, strongly associated with tobacco use. This condition involves a panarteritis, affecting small and medium-sized arteries, veins, and nerves in the extremities. It is most commonly seen in young male smokers, typically between the ages of 20 and 40 years.

About

• Demographics: Most common in young male smokers aged 20-40 years, with peak incidence in the 25-35 age range.
• Geographic Distribution: Higher prevalence among individuals from the Mediterranean, Middle East, and North Africa.
• Presentation: Characterized by pain and claudication in the limbs, leading to progressive ischaemia and possible gangrene if untreated.

Aetiology

• Inflammatory Disease: A non-atherosclerotic inflammatory process involving the small and medium-sized vessels of the distal extremities.
• Distinct from Atherosclerosis: Unlike atherosclerosis, it affects younger individuals and lacks typical atherosclerotic plaques.
• Tobacco Use: Strongly associated with tobacco use; the disease often progresses with continued smoking and may stabilize or improve with cessation.
• Affected Structures: Involves arteries, veins, and peripheral nerves, leading to deep vein thrombosis, arterial occlusions, and distal neuropathy.

Clinical Features

• Claudication: Pain in the legs, arms, hands, and feet during exertion due to reduced blood flow, which can progress to rest pain as the disease advances.
• Rest Pain: Severe pain at rest due to critical limb ischaemia, often requiring medical or surgical intervention.
• Digital Ischemia: Progressive ischaemia of the fingers and toes, leading to ulcers, gangrene, and possible loss of digits.
• Pulse Examination: Proximal pulses (e.g., brachial and popliteal) may be preserved, but distal pulses (e.g., radial, ulnar, posterior tibial) often disappear first.
• Phlebitis Migrans: Superficial vein inflammation that can migrate, presenting as tender nodules along the veins.

Shionoya Clinical Criteria

The Shionoya criteria help in diagnosing Thromboangiitis Obliterans:

• 1. History of smoking or tobacco use.
• 2. Onset before the age of 50 years.
• 3. Infrapopliteal arterial occlusions (disease involving the arteries below the knee).
• 4. Presence of either upper limb involvement or migratory phlebitis.
• 5. Absence of other atherosclerotic risk factors (e.g., diabetes, hyperlipidemia) apart from smoking.

Investigations

• Laboratory Tests: ESR and CRP levels are typically normal, distinguishing it from other inflammatory vasculitides.
• Angiography: Gold standard for diagnosis, revealing "corkscrew" or "tree-root" appearance of small distal arteries and segmental occlusions.
• Doppler Ultrasound: May be used to assess blood flow in the extremities and detect occlusions, but angiography is more definitive.

Management

• Tobacco Cessation: The most critical aspect of management; complete cessation can halt the progression of the disease and may improve symptoms.
• Medical Therapy:
  • Antiplatelet agents like aspirin may be used but have limited effectiveness in halting disease progression.
  • Calcium channel blockers (e.g., nifedipine) may be tried to relieve vasospasm, though their benefit is often temporary.
  • Prostacyclin analogs like iloprost (IV infusions) may help reduce symptoms and improve distal circulation in some patients.
• Surgical Interventions:
  • Sympathectomy: May be considered to provide temporary pain relief in severe cases, though it does not alter disease progression.
  • Amputation: Required for those with non-healing ulcers, severe rest pain, or gangrene. Often necessary in patients who continue to smoke.
  • Reconstructive Surgery: Rarely possible due to the small vessel involvement and distal nature of the occlusions.
• Supportive Care: Includes pain management, wound care for ulcers, and management of neuropathic pain.

Prognosis

• Improves with Smoking Cessation: Disease progression can be arrested or slowed with complete tobacco cessation, leading to symptom improvement in many cases.
• Poor Outcome with Continued Smoking: Progression to gangrene and limb loss is common if the patient continues to smoke.
• Quality of Life: Chronic pain, digital loss, and need for amputations can significantly impact the quality of life, emphasizing the importance of early intervention and support.

References

• Thromboangiitis Obliterans (Buerger’s Disease)
• Current Concepts in the Pathogenesis and Management of Buerger's Disease
• Medscape: Buerger's Disease Overview