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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

SURGERY

  • Amputation may be necessary in up to 50% of patients

  • Thoracoscopic sympathectomy ...

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