Buerger’s Disease (Thromboangiitis Obliterans)

KEY FEATURES

ESSENTIALS OF DIAGNOSIS

• An inflammatory vasculopathy (arteries and veins) that predominantly involves the hands and feet and is invariably associated with smoking

• Digital ulcers may be present

• Men younger than 40 years

• Thrombophlebitis may be the first sign of the disease, especially phlebitis migrans

• Angiography shows disease of the small and median arteries with corkscrew collaterals (Martorell’s sign); large arteries are unaffected

• Laboratory evidence of HLA antibodies, increased complement activity, and anticollagen antibodies

GENERAL CONSIDERATIONS

• The cause of Buerger’s disease is unknown, but cigarette smoking plays a critical role in its genesis

• It occurs primarily in young men (4:1 ratio) and almost exclusively in smokers, ex-smokers, and passive smokers

• The inflammatory activity is seen in both arteries and veins with migratory thrombophlebitis

• Ischemia most often involves the digits

• Buerger’s disease is more common in the eastern Mediterranean and Asia

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

• The first sign is often thrombophlebitis in an extremity

• Inflammation of the digits with pain and swelling

PHYSICAL EXAM FINDINGS

• Pulse deficits in distal extremities

• Signs of ischemia, such as ulcers

DIFFERENTIAL DIAGNOSIS

• Atherosclerosis obliterans

• Other vasculitides

DIAGNOSTIC EVALUATION

LABORATORY TESTS

• Acute phase reactants such as the sedimentation rate, C-reactive protein, and various autoantibodies are usually normal

• Increased anti-endothelial cell antibodies

• Increased cellular immunity to collagen types 1 and 3

• Anticardiolipin antibodies increased in some

SHIONOYA CRITERIA

• History of smoking

• Age of onset < 50 years

• Infrapopliteal arterial occlusions

• Upper limb involvement or phlebitis migrans

• Absence of other atherosclerosis risk factors

DIAGNOSTIC PROCEDURES

• Angiography is usually diagnostic:

  • – Segmental lesions in otherwise normal arteries

  • – Small- to medium-sized arteries involved with more disease distally

  • – Corkscrew collaterals (Martorell’s sign), but these are not pathognomonic

• Biopsy may be necessary in some and shows a diffuse vasculitis involving arteries and veins with preservation of the internal elastic lamina

TREATMENT

CARDIOLOGY REFERRAL

• Suspected thromboembolic disease

HOSPITALIZATION CRITERIA

• Complications, such as infection

• Gangrene

MEDICATIONS

• Smoking cessation is the cornerstone of therapy (including marijuana, nicotine patches, and chewing tobacco)

• IV prostacyclin analogs such as iloprost is the treatment of choice

• Bosentan may help

• SC treprostinil may help

• Other vasodilators such as sildenafil, calcium antagonists, and alpha blockers have not been studied

THERAPEUTIC PROCEDURES

• Hyperbaric oxygen may help ulcer healing

• Stem cells to stimulate angiogenesis is being studied

• Spinal cord stimulation may help