What is Buerger's Disease and How is it Treated?
Buerger's disease (thromboangiitis obliterans) is a nonatherosclerotic inflammatory vasculitis affecting small- and medium-sized arteries and veins in the distal extremities of young tobacco smokers, and complete tobacco cessation is the only definitive treatment that halts disease progression and prevents amputation. 1, 2
Definition and Pathophysiology
Buerger's disease is a segmental thrombotic occlusive process that behaves like a vasculitis, causing inflammation in arteries of all sizes and superficial veins, almost exclusively in young tobacco smokers under age 50. 1 The disease is characterized by intensely cellular vessel wall inflammation, giant cell foci, and hypercellular thrombi, but with preservation of the elastic lamina and overall vascular wall architecture. 3 While the exact cause remains unknown, most investigators believe it is an immune-mediated endarteritis triggered when nicotine is present, with linear deposition of immunoglobulins and complement factors along the elastic lamina. 3, 4
Clinical Presentation and Diagnosis
Patients are typically young males (onset before age 45-50) who are inveterate tobacco smokers presenting with distal extremity ischemia, ischemic ulcers, or frank gangrene of the toes or fingers. 1, 3 The incidence is approximately 12.6 per 100,000 in North America. 1
Diagnostic Criteria (Shionoya's Clinical Criteria)
To confirm diagnosis, verify all five of the following criteria: 2, 3, 4
- History of smoking or tobacco abuse
- Age of onset less than 45-50 years
- Infrapopliteal arterial occlusions with sparing of proximal vasculature
- Either upper limb involvement (Raynaud's syndrome or digital ulceration) or phlebitis migrans
- Absence of atherosclerotic risk factors other than smoking (must exclude arteriosclerosis, diabetes, true arteritis, proximal embolic source, and hypercoagulable states)
Imaging Findings
Arteriography classically demonstrates "corkscrew," "spider legs," or "tree roots" collateral vessels representing pathologically dilated vasa vasorum—these findings are suggestive but not pathognomonic and should not be used alone for diagnosis. 1, 2, 4
Treatment Approach
Primary Treatment: Absolute Tobacco Cessation
Complete and permanent cessation of all tobacco use is the only definitive treatment that halts disease progression and prevents amputation. 2 Tobacco abstinence generally results in disease quiescence and remains the mainstay of treatment. 3 Each clinician should advise patients to stop smoking and offer comprehensive cessation interventions including behavior modification therapy, nicotine replacement therapy, or bupropion. 2
Initial Conservative Management
Initiate antiplatelet therapy (such as aspirin) to reduce risk of myocardial infarction, stroke, or vascular death. 2 The initial management should be conservative: claudicants should be encouraged to walk, whereas patients with critical ischemia should be admitted for bed rest. 4
Pharmacological Treatment for Severe Disease
For patients with severe complications such as ischemic ulcers or rest pain who cannot undergo revascularization: 5
Intravenous iloprost (prostacyclin analogue) is more effective than aspirin for eradicating rest pain and healing ischemic ulcers (moderate-certainty evidence). 5 Specifically, intravenous iloprost improved ulcer healing (RR 2.65) and helped eradicate rest pain after 28 days (RR 2.28) compared to aspirin. 5
Oral iloprost is not more effective than placebo for healing ischemic ulcers, eradicating rest pain, or reducing amputation rates at six months (moderate-certainty evidence). 5
There is no clear difference between prostacyclin analogues (iloprost and clinprost) and prostaglandin analogue (alprostadil) for healing ulcers and relieving pain in severe Buerger's disease (very low to low-certainty evidence). 5
Surgical Options
Bypass grafting is seldom an option because the distal location of lesions leaves little to bypass due to lack of suitable target vessels or venous conduits. 6, 4 When vascular reconstruction is attempted (mainly femorodistal bypasses), bypass patency rates are suboptimal, though limb salvage rates are satisfactory because patent grafts, even over a short period, are sufficient to allow healing of ulcers. 4
Lumbar or thoracic sympathectomy may be considered in select cases. 7
Emerging Therapies
Therapeutic angiogenesis techniques using DNA plasmids, viral gene constructs, and cell-based preparations are under investigation for patients with critical limb ischemia who have no revascularization options, though these remain experimental. 6
Important Clinical Considerations
Common pitfall: Do not use anticoagulants, antiplatelet agents beyond standard therapy, or antifibrinolytic agents for venous thrombosis in Buerger's disease—venous thrombi adhere to the vessel wall and do not result in emboli, and pulmonary embolism is rare despite high frequency of venous thrombosis. 4 Additionally, avoid these agents due to the possibility of coexisting pulmonary arterial aneurysm, which might result in fatal bleeding. 4
Periodontal disease association: More than half of Buerger's disease patients suffer from severe periodontitis, and improvement of periodontal care may improve clinical symptoms in addition to smoking cessation. 7
Disease progression: Without tobacco cessation, patients often suffer from severe ischemic pain and tissue loss culminating in minor and major limb amputation, though mortality is not increased. 3