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BUERGER'S DISEASE (THROMBOANGIITIS OBLITERANS)

Introduction

Buerger’s disease was first described as a “presenile spontaneous gangrene” by Dr Felix von Winiwarter in 1879. It was later termed thromboangiitis obliterans by Dr Leo Buerger in 1908. It is an inflammatory disorder that affects small- and medium-sized vessels in the extremities, particularly the hands and feet. All layers of the vessel wall become acutely inflamed—which is why Buerger’s disease is classified as a vasculitis—and this leads to swelling and blockage by blood clots (thrombi). Although similar to peripheral arterial disease (PAD), Buerger’s disease differs in that it is nonatherosclerotic; Peripheral artery disease is caused by plaque buildup in the arteries. Several systems have been developed for the diagnosis and staging of Buerger’s disease: Shionoya’s criteria, Papa’s scoring system, Olin’s criteria, Rutherford classification, and Leriche-fontaine classification. None is accepted as definitive.

Pathophysiology

  • There are three phases of Buerger’s disease:
    • Acute: polymorphonuclear leukocytes (PMNs) are predominant at the site of inflammation, and they may form microabscesses within the thrombus
    • Subacute: PMNs are surrounded by a granulomatous inflammation, which may lead to formation of a new path (recanalization) through the thrombus
    • Chronic: a mature thrombus with vascular fibrosis is evident
  • The normal structure of the affected vessel, and particularly the internal elastic lamina, remains intact in all three phases.
  • Buerger's disease is a vasculitis that attacks the small and medium vessels of the extremities (arteries and veins).3

Incidence and Related Conditions

  • Buerger’s disease is found globally, but is more prevalent in the Middle East and Far East than in North America and Western Europe
  • In the United States, incidence is estimated to be 0.02% of the population.
  • The cause of Buerger’s disease is unknown, although there is a powerful association with heavy tobacco use, which may involve:
    • Allergic-hyperergic/immune reaction to antigens in tobacco
    • Presence of antielastin antibodies
    • Decreased activity of proteinase inhibitors
    • Genetic propensity
  • Other related conditions (hypothetical causes) of Buerger’s disease include bacterial infection (poor oral hygiene) and coagulation system abnormalities
  • Buerger's disease presents most often in the lower extremities with the upper extremity involved in 15-20% of the cases.3

Differential Diagnosis

  • Antiphospholipid syndrome
  • Arteriosclerosis
  • Atherosclerosis obliterans
  • Behcet's disease(deep vein thrombophlebitis is suggestive, and unusual in Buerger’s disease)
  • Drug-induced vasculitides  
  • Hypothenar hammer syndrome
  • Infectious endocarditis  
  • Mixed connective tissue disease
  • Occlusive vasculopathy due to systemic lupus erythematosus or generalized systemic sclerosis
  • Peripheral embolism and atherothrombotic embolism
  • Persistent sciatica artery
  • Popliteal entrapment syndrome
  • Rheumatoid arthritis 
  • Takayasu arteritis
  • Thoracic outlet syndrome
  • Traumatic arterial thrombosis
  • Trench foot/immersion foot syndrome
  • Vasculitis, other forms
ICD-10 Codes

  • BUERGER'S DISEASE (THROMBOANGIITIS OBLITERANS)

    Diagnostic Guide Name

    BUERGER'S DISEASE (THROMBOANGIITIS OBLITERANS)

    ICD 10 Diagnosis, Single Code, Left Code, Right Code and Bilateral Code

    DIAGNOSISSINGLE CODE ONLYLEFTRIGHTBILATERAL (If Available)
    BUERGER'S DISEASEI73.1   

    ICD-10 Reference

    Reproduced from the International statistical classification of diseases and related health problems, 10th revision, Fifth edition, 2016. Geneva, World Health Organization, 2016 https://apps.who.int/iris/handle/10665/246208

Clinical Presentation Photos and Related Diagrams
Buerger Disease or Thromboangiitis Obliterans
  • Fingertip necrosis associated with Buerger disease
    Fingertip necrosis associated with Buerger disease
Symptoms
Extreme limb pain while at rest
Skin ulcerations
Gangrene of the fingers and toes
Bilateral evidence of disease
Coldness, numbness, or tingling (parestesias) of hands or feet
Intermittent claudication (pain in hands and feet when exercising)
Periodontal disease
History of heavy tobacco use
Typical History

Early in the disease course, the patient will present with the hands affected; the disorder may eventually extend to the arms. Two or more limbs are almost always involved. The patient is usually male and aged 25–35 years. Initial complaints include cramping of the arch of the foot and calves, pain in the extremities while at rest, tingling, numbness, pale skin, and poor reaction to cold temperatures. The patient may have a history of high cholesterol, high blood pressure, or diabetes. Importantly, the patient is likely to be a tobacco user.

Positive Tests, Exams or Signs
Work-up Options
Treatment Options
Conservative

Treatment is symptomatic and supportive, but must include immediate and complete cessation of all tobacco use; patients who continue to use tobacco risk amputation

  • Complete remission is possible with cessation of tobacco use; however, one study showed that decreasing the number of cigarettes had no effect on disease outcome
  • Patient education and psychological assistance can help patients quit
  • Medications that can improve pain-free walking distance: vasodilators, pentoxyfylline, and cilostazol
  • Medications helpful for intermittent claudication: naftidrofuryl, levocarnitine, arginine, buflomedil, ketanserin, niacin, and lovastatin
  • Rheumatology consult to evaluate the differential diagnosis
  • Vascular surgery consult but angiogram should be done to identify any potential opportunities for vascular bypass grafting
Operative
  • Surgical revascularization is rarely possible because vascular damage is diffuse
  • Bypass vascular grafting
  • Arterialization of the venous system with or without vein grafts3
  • Sympathectomy may provide short-term pain relief and promote ulcer healing in some patients
Outcomes
  • Smoking cessation can result in dramatic improvement and remission
    • Only 43–70% of patients give up smoking
    • Smoking duration has a significant relationship with adverse outcome
  • Surgical revascularization (rare): patency rates up to 67% in patients who quit smoking
  • Sympathectomy: long-term benefits have not been demonstrated
Key Educational Points
  • In patients with severe ischemia of the upper limbs, thoracoscopic sympathectomy controls symptom and maximizes tissue salvage while being minimally invasive with few complications. However, there are no long-term data
  • Involvement of other vascular areas (eg, celiac arteries, large vessels, coronary arteries) is rarely associated with Buerger’s disease
  • Consider occupational hazards, such as use of vibratory tools, if involvement is isolated to the upper extremity
  • In the upper extremity, the ulnar artery is most commonly affected
  • Four clinical criteria:
    • Rest pain or ulceration in the hands or feet before age 50 years
    • Tobacco use/smoking history
    • Tests (usually an angiogram) indicating blockage of arteries
    • No other causes of artery blockage or clot development
  • Complete serologic profile to exclude other disorders that mimic Buerger’s disease
  • Echocardiography and arteriography to exclude a proximal source of emboli
  • Histopathology: highly inflammatory thrombus infiltrated with PMNs and multinucleated giant cells, affecting both arteries and veins
  • Arteriography (at least one of the following is required):
    • Multiple segmental occlusions of distal arterial sections (distally from the elbow and knee)
    • Chronic vascular occlusion due to secondary thrombosis
    • Absence of atherosclerotic lesions, such as calcification of vascular walls
    • Tapering and abrupt obstruction of vessels, a twisting course of the involved vessels “Corkscrew” or “tree-root” collaterals
References

New Articles

  1. Vijayakumar A, Tiwari R, Kumar Prabhuswamy V. Thromboangiitis obliterans (Buerger’s disease)—current practices. Int J Inflamm 2013;156905. PMID: 24102033
  2. Małecki R, Kluz J, Przeździecka-Dołyk J, Adamiec R. The pathogenesis and diagnosis of thromboangiitis obliterans: is it still a mystery? Adv Clin Exp Med 2015;24(6):1085-97. PMID: 26771983
  3. Higgins JP, McClinton MA. Vascular insufficiency of the upper extremity. J Hand Surg 2010;35A:1545-1553. PMID: 20807633

Reviews

  1. Rivera-Chavarría IJ, Brenes-Gutiérrez JD. Thromboangiitis obliterans (Buerger’s disease). Ann Med Surg 2016;7:79-82. PMID: 27144003

Classics

  1. Orban F. New trends in the treatment of thromboangeiosis (Buerger's disease). Ann R Coll Surg Engl 1961;28:69-100. PMID: 13731098
  2. Schatz IJ, Fine G, Eyler WR. Thromboangiitis obliterans. Br Heart J 1966;28(1):84-91. PMID: 5904753

Other

  1. National Organization for Rare Disorders. Buerger’s disease. 2007. Accessed October 16, 2016 at https://rarediseases.org/rare-diseases/buergers-disease/
  2. University of California–Davis Health System. Buerger’s disease. 2016. Accessed October 16, 2016 at www.ucdmc.ucdavis.edu/vascular/diseases/buergers_disease.html
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