29th January 2024

Thromboangiitis obliterans (Buerger disease)

Background

Epidemiology 

Most commonly affects adult males with significant history of cigarette smoking (most significant risk factor). Onset is usually before the age of 45 years. Prevalence is up to 20 cases per 100,000 individuals; however, it is more common in Asia and less common among African Americans. 

Aetiology

The exact cause of Buerger’s disease is unknown; however, tobacco use, particularly cigarette smoking, is an overwhelming predisposing factor for disease. It is thought that the disease is triggered by chemicals in tobacco which irritate the lining of blood vessels. 

Pathophysiology 

Thromboangiitis obliterans affects both small and medium-sized arteries and veins. It localises in the distal arteries of the upper and lower extremities.

Inflammation of the tunica intima with neutrophilic infiltration and micro-abscess formation can spread to the tunica media with the internal elastic lamina usually intact. The development of cell-rich, inflammatory thrombi in the lumen occludes the vessel. This is followed by reorganisation of the thrombus and perivascular fibrosis. Contiguous extension of the inflammatory process to the adjacent structures results in the encasement of the artery, vein and nerve in a fibrous sheath. 

Clinical features

Patients may present with acute limb ischaemia or symptoms of peripheral vascular disease. Early manifestations include superficial thrombophlebitis (often migratory) with tender nodules along the course of the vein, intermittent claudication and Raynaud phenomenon. Later stages of the disease may present with severe rest pain, cold peripheral extremities, trophic nail changes, ulceration or gangrene of the digits, which may autoamputate. Symptoms typically begin in the extremities but radiate more to the proximal parts of the body. Patients typically have normal brachial and popliteal pulses but poor or absent distal pulsations. 

Diagnosis

Typical laboratory findings include normal ESR and CRP levels with absent autoantibodies and normal hypercoagulability screen. A decreased ankle-brachial index is suggestive of the disease. Arteriography is the imaging modality of choice and can show extensive arterial occlusive disease with corkscrew collateral vessels around the site of occlusion. Confirmation of diagnosis requires excisional skin biopsy; however, this is rarely performed.

Treatment 

The most important management is the complete cessation of smoking and tobacco products. Conservative management for Raynaud’s phenomenon, such as protecting fingers and toes from the cold is important. Ulcerations may need debridement, antibiotic treatments, and amputation may be required in extreme cases. 

Medications such as Iloprost, calcium channel blockers (nifedipine, amlodipine) and hyperbaric oxygen therapy can at times be used. 

References

• About vasculitis, Vasculitis UK. Available at: https://www.vasculitis.org.uk/about-vasculitis [Accessed 28th January 2022].
• Buerger’s Disease, Johns Hopkins Vasculitis Center. Available at: https://www.hopkinsvasculitis.org/types-vasculitis/buergers-disease/ [Accessed 16th January 2022].
• Knipe. H., 2021. Buerger disease, Radiopaedia. Available at: https://radiopaedia.org/articles/buerger-disease?lang=gb [Accessed 3rd February 2022].
• Vasculitides, Amboss. Available at: https://www.amboss.com/us/knowledge/Vasculitides/ [Accessed 17th January 2022].