What is Buerger's disease, a condition affecting young to middle-aged men who are heavy smokers?

Buerger's Disease (Thromboangiitis Obliterans)

Buerger's disease is a nonatherosclerotic inflammatory vasculitis that causes segmental thrombotic occlusions of small- and medium-sized arteries and veins in the distal extremities, almost exclusively affecting young tobacco smokers under age 50. 1

Pathophysiology and Demographics

• This is an inflammatory occlusive vasculitis that behaves like an arterial obliterative and thrombotic process, affecting arteries of all sizes as well as superficial veins. 1

• The disease predominantly affects young male tobacco smokers, typically less than 45-50 years old at onset, with a near-universal finding of heavy tobacco use. 1

• The incidence is approximately 12.6 per 100,000 in North America. 1

• Males are affected more commonly than females (55 males to 3 females in one surgical series). 2

Clinical Manifestations

Early Symptoms

• Migratory superficial thrombophlebitis is a common early finding, characterized by recurrent episodes affecting superficial veins of extremities. 1

• Paresthesias, including numbness or tingling in affected digits, occur early in the disease course. 1

Progressive Ischemic Symptoms

• Intermittent claudication develops in 23% of patients, with progressive worsening of exercise-induced pain. 1, 2

• Rest pain occurs in 13% of patients, manifesting as severe pain in affected extremities even without activity. 1, 2

• Ischemic ulcers or frank gangrene of the toes or fingers develop in 64% of patients, representing advanced disease. 1, 2

• Upper limb involvement, including cyanosis, paleness, and gangrene, is obvious in 84% of patients. 2

Disease Distribution

• While classically affecting distal extremities, arterial lesions can extend to the femoral arteries in 25% of cases, to the iliac arteries in 8%, and to the abdominal aorta and/or visceral arteries in 6% of cases. 2

Diagnostic Criteria

The diagnosis is based on Shionoya's clinical diagnostic criteria, which must all be fulfilled: 2, 3

1. History of smoking (or tobacco use)
2. Onset before age 50 years
3. Presence of infrapopliteal arterial occlusions
4. Either upper limb involvement or phlebitis migrans
5. Absence of atherosclerotic risk factors other than smoking

Imaging Characteristics

• Arteriography classically demonstrates "corkscrew," "spider legs," or "tree roots" collateral vessels representing pathologically dilated vasa vasorum. 1, 4

• These angiographic findings are suggestive but not pathognomonic and should not be used alone for diagnosis. 4, 3

Treatment

Absolute Tobacco Cessation (Primary Treatment)

Complete and permanent cessation of all tobacco use is the only definitive treatment that halts disease progression and prevents amputation. 4

• Continued smoking is directly linked to disease advancement. 5

• Each clinician should advise patients to stop smoking and offer comprehensive cessation interventions including behavior modification therapy, nicotine replacement therapy, or bupropion. 4

• Critical caveat: Substitution of smokeless tobacco for cigarettes does not prevent limb loss and can lead to progressive disease requiring bilateral below-knee amputations. 6

Medical Management

• Initiate antiplatelet therapy to reduce risk of myocardial infarction, stroke, or vascular death. 4

• Infusion of iloprost has demonstrated some efficacy in patients with ischemic lesions. 7

Conservative Management

• Claudicants should be encouraged to walk, whereas patients with critical ischemia should be admitted for bed rest in the hospital. 3

• Local wound care is the main component of therapeutic management for patients with ischemic lesions. 7

Surgical Options

• Bypass grafting is seldom an option, as the location of lesions distally leaves little to bypass because of lack of target vessels. 3

• When performed, femorodistal bypasses show suboptimal patency rates but satisfactory limb salvage rates, as patent grafts even over a short period allow healing of ulcers. 3

• Lumbar sympathectomy was performed in 33 patients and thoracic sympathectomy in 8 patients in one surgical series. 2

Amputation Rates

• Minor limb amputations are required in 20% of patients, and major limb amputations in 4% of patients. 2

Associated Conditions

• More than half of Buerger's disease patients suffer from severe periodontitis (grades B, C, or D), suggesting that improvement of periodontal care could potentially improve clinical symptoms. 2