• Clinical science

Thromboangiitis obliterans (Buerger disease)

Abstract

Thromboangiitis obliterans (TAO, Buerger's disease) is an inflammatory, non-atherosclerotic, vaso-occlusive disease of both small and medium-sized arteries as well as veins in the upper and lower limbs. TAO typically affects male adults and is strongly associated with cigarette smoking. In susceptible individuals, smoking causes inflammation of the tunica intima of small vessels by an unknown mechanism, which results in thrombotic occlusion of the vessel. Patients initially present with a classic triad of intermittent claudication, Raynaud's phenomenon, and migratory thrombophlebitis. Eventually, critical limb ischemia develops and the patient presents with rest pain, absent pulse in the extremities, and/or digital gangrene. Ultrasonography and arteriography are used to localize the site of occlusion and differentiate TAO from other causes of peripheral artery disease. The most important therapeutic measure is the complete cessation of smoking. Additionally, prostaglandin analogs (e.g., iloprost) may be used to improve blood flow and decrease rest pain. Patients with TAO who develop digital gangrene require amputation.

Epidemiology

  • Prevalence: 13–20 cases per 100,000 individuals
  • Sex: > (3:1)
  • Age of onset: 20–45 years
  • Ethnicity: Ashkenazi Jews, Indians, Koreans, Japanese

References:[1][2][3]

Epidemiological data refers to the US, unless otherwise specified.

Pathophysiology

  • Thromboangiitis obliterans is an inflammatory, non-atherosclerotic vasculitic disease that affects both small and medium-sized arteries as well as the veins.
  • Smoking is the single most important risk factor for TAO.
  • Stages of TAO
    1. Inflammation of the tunica intima; with neutrophilic infiltration and microabscess formation (endarteritis) → development of cell-rich, inflammatory thrombi; in the lumen → occlusion of the vessel
    2. Mononuclear cells, fibroblasts, and giant cells replace neutrophils
    3. Reorganization of the thrombus and perivascular fibrosis
    4. Contiguous extension of inflammatory process to the adjacent vein and nerve, resulting in the encasement of the artery, vein, and nerve in a fibrous sheath
  • Disease localization: distal arteries of the upper and lower extremities

References:[1][3]

Clinical features

Patients may present with acute limb ischemia and/or symptoms of chronic peripheral artery disease (see “Clinical features” of peripheral artery disease).

  • Early manifestations
  • Late manifestations
    • Rest pain
    • Cool peripheral extremities
    • Trophic nail changes
    • Ulceration and/or gangrene of fingertips and/or toes
    • Normal brachial and popliteal pulses but poor/absent radial, ulnar, anterior tibial, posterior tibial and/or dorsal pedis pulsations

References:[4][3]

Diagnostics

  • Laboratory findings
    • ESR and CRP are within normal limits.
    • Markers of autoimmune disease (e.g., ANA, RF) are absent and the hypercoagulability screen is normal.
  • Imaging
    • Doppler ultrasound (initial imaging): ankle-brachial index
    • Arteriography (imaging modality of choice): non-atherosclerotic, smooth, tapering, segmental lesions that occlude distal vessels of extremities with corkscrew-shaped collateral vessels around the site of occlusion
  • Although confirmation of the diagnosis requires excisional skin biopsy, biopsies are rarely performed.

ESR and CRP remain within normal limits!
References:[4][2]

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

  • General measures
    • Complete cessation of smoking and the use of tobacco products is the single most important therapeutic measure
    • Protection of fingers and toes from cold to prevent Raynaud's phenomenon
  • Medical therapy
  • Surgical therapy
    • Revascularization procedures (e.g., bypass grafting, angioplasty) usually cannot be performed because, typically, distal small and medium-sized arteries are involved.
    • Patients with ulcers may require debridement and treatment with antibiotics.
    • Patients who develop gangrene in the extremities will require amputation.


References:[1][2][3]

  • 1. Kumar V, Abbas AK, Aster JC. Robbins & Cotran Pathologic Basis of Disease. Philadelphia, PA: Elsevier Saunders; 2014.
  • 2. Olin JW. Thromboangiitis obliterans (Buerger's disease). In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/thromboangiitis-obliterans-buergers-disease. Last updated October 30, 2015. Accessed February 13, 2017.
  • 3. Nassiri N. Thromboangiitis Obliterans (Buerger Disease). In: Thromboangiitis Obliterans (Buerger Disease). New York, NY: WebMD. http://emedicine.medscape.com/article/460027. Updated December 19, 2016. Accessed February 13, 2017.
  • 4. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. New York, NY: McGraw-Hill Education; 2015.
  • Herold G. Internal Medicine. Cologne, Germany: Herold G; 2014.
last updated 11/02/2018
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