Thromboangiitis obliterans

Last updated
Thromboangiitis obliterans
Other namesBuerger disease, Buerger's disease, Winiwarter-Buerger disease, presenile gangrene [1]
M.Buerger 1.JPG
Complete occlusion of the right and stenosis of the left femoral artery as seen in a case of thromboangiitis obliterans
Specialty Cardiology, rheumatology   OOjs UI icon edit-ltr-progressive.svg

Thromboangiitis obliterans, also known as Buerger disease (English /ˈbɜːrɡər/ ; German: [ˈbʏʁɡɐ] ) or Winiwarter-Buerger disease, is a recurring progressive inflammation and thrombosis (clotting) of small and medium arteries and veins of the hands and feet. It is strongly associated with use of tobacco products, [2] primarily from smoking, but is also associated with smokeless tobacco. [3] [4]


Signs and symptoms

There is a recurrent acute and chronic inflammation and thrombosis of arteries and veins of the hands and feet. The main symptom is pain in the affected areas, at rest and while walking (claudication). [1] The impaired circulation increases sensitivity to cold. Peripheral pulses are diminished or absent. There are color changes in the extremities. The colour may range from cyanotic blue to reddish blue. Skin becomes thin and shiny. Hair growth is reduced. Ulcerations and gangrene in the extremities are common complications, often resulting in the need for amputation of the involved extremity. [5]


There are characteristic pathologic findings of acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still largely unknown, but smoking and tobacco consumption are major factors associated with it. It has been suggested that the tobacco may trigger an immune response in susceptible persons or it may unmask a clotting defect, either of which could incite an inflammatory reaction of the vessel wall. [6] This eventually leads to vasculitis and ischemic changes in distal parts of limbs.[ citation needed ]

A possible role for Rickettsia in this disease has been proposed. [7]


A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. The commonly followed diagnostic criteria are outlined below although the criteria tend to differ slightly from author to author. Olin (2000) proposes the following criteria: [8]

  1. Typically between 20 and 40 years old and male, although recently females have been diagnosed. [9]
  2. Current (or recent) history of tobacco use.
  3. Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as ultrasound.
  4. Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.
  5. Exclusion of a proximal source of emboli by echocardiography and arteriography.
  6. Consistent arteriographic findings in the clinically involved and noninvolved limbs.

Buerger's disease can be mimicked by a wide variety of other diseases that cause diminished blood flow to the extremities. These other disorders must be ruled out with an aggressive evaluation, because their treatments differ substantially from that of Buerger's disease, for which there is no treatment known to be effective.[ citation needed ]

Some diseases with which Buerger's disease may be confused include atherosclerosis (build-up of cholesterol plaques in the arteries), endocarditis (an infection of the lining of the heart), other types of vasculitis, severe Raynaud's phenomenon associated with connective tissue disorders (e.g., lupus or scleroderma), clotting disorders or the production of clots in the blood.[ citation needed ]

Angiograms of the upper and lower extremities can be helpful in making the diagnosis of Buerger's disease. In the proper clinical setting, certain angiographic findings are diagnostic of Buerger's. These findings include a "corkscrew" appearance of arteries that result from vascular damage, particularly the arteries in the region of the wrists and ankles. Collateral circulation gives "tree root" or "spider leg" appearance. [1] Angiograms may also show occlusions (blockages) or stenosis (narrowings) in multiple areas of both the arms and legs. Distal plethysmography also yields useful information about circulatory status in digits. To rule out other forms of vasculitis (by excluding involvement of vascular regions atypical for Buerger's), it is sometimes necessary to perform angiograms of other body regions (e.g., a mesenteric angiogram).[ citation needed ]

Skin biopsies of affected extremities are rarely performed because of the frequent concern that a biopsy site near an area poorly perfused with blood will not heal well.[ citation needed ]


The cause of the disease is thought to be autoimmune in nature and heavily linked to tobacco use in patients with Buerger's as primary disease.[ clarification needed ]


Smoking cessation has been shown to slow the progression of the disease and decrease the severity of amputation in most patients, but does not halt the progression.[ citation needed ]

Treatment by 100% hyperbaric oxygen. Barokomora Nis hyperbaric center.JPG
Treatment by 100% hyperbaric oxygen.

In acute cases, drugs and procedures which cause vasodilation are effective in reducing pain experienced by patient. For example, prostaglandins like Limaprost [10] are vasodilators and give relief of pain, but do not help in changing the course of disease. Epidural anesthesia and hyperbaric oxygen therapy also have vasodilator effect. [1] There is moderate certainty evidence that intravenous iloprost (prostacyclin analogue) is more effective than aspirin for relieving rest pain and healing ischemic ulcers. [11] No difference have been detected between iloprost or clinprost (prostacyclin) and alprostadil (prostaglandin analogue) for relieving pain and healing ulcers. [11]

In chronic cases, lumbar sympathectomy may be occasionally helpful. [12] It reduces vasoconstriction and increases blood flow to limb. It aids in healing and giving relief from pain of ischemic ulcers. [1] Bypass can sometimes be helpful in treating limbs with poor perfusion secondary to this disease. Use of vascular growth factor and stem cell injections have been showing promise in clinical studies. There may be a benefit of using bone marrow-derived stem cells in healing ulcers and improving pain-free walking distance, but larger, high-quality trials are needed. [13] Debridement is done in necrotic ulcers. In gangrenous digits, amputation is frequently required. Below-knee and above-knee amputation is rarely required. [1]

Streptokinase has been proposed as adjuvant therapy in some cases. [14]

Despite the clear presence of inflammation in this disorder, anti-inflammatory agents such as corticosteroids have not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low dosage intermittent form. Similarly, strategies of anticoagulation have not proven effective. physical therapy: interferential current therapy to decrease inflammation.[ citation needed ]


Buerger's is not immediately fatal. Amputation is common and major amputations (of limbs rather than fingers/toes) are almost twice as common in patients who continue to smoke. Prognosis markedly improves if a person quits smoking. Female patients tend to show much higher longevity rates than men. The only known way to slow the progression of the disease is to abstain from all tobacco products.[ citation needed ]


Buerger's is more common among men than women. Although present worldwide, it is more prevalent in the Middle East and Far East. [15] Incidence of thromboangiitis obliterans is 8 to 12 per 100,000 adults in the United States (0.75% of all patients with peripheral vascular disease). [15]


Buerger's disease was first described by Felix von Winiwarter in 1879 in Austria. [16] It was not until 1908, however, that the disease was given its first accurate pathological description, by Leo Buerger at Mount Sinai Hospital in New York City, who referred to the condition as "presenile spontaneous gangrene". [17]

Notable people affected

As reported by Alan Michie in God Save the Queen, published in 1952 (see pages 194 and following), King George VI was diagnosed with the disease on 12 November 1948. Both legs were affected, the right more seriously than the left. The king's doctors prescribed complete rest and electric treatment to stimulate circulation, but as they were either unaware of the connection between the disease and smoking (the king was a heavy smoker) or unable to persuade the king to stop smoking, the disease failed to respond to their treatment. On 12 March 1949, the king underwent a lumbar sympathectomy, performed at Buckingham Palace by James R. Learmonth. The operation, as such, was successful, but the king was warned that it was a palliative, not a cure, and that there could be no assurance that the disease would not grow worse. From all accounts, the king continued to smoke.[ citation needed ]

The author and journalist John McBeth describes his experiences of the disease, and treatment for it, in the chapter "Year of the Leg" in his book Reporter: Forty Years Covering Asia. [18]

Philippine president Rodrigo Duterte disclosed in 2015 that he has Buerger's disease. [19]

Related Research Articles

<span class="mw-page-title-main">Amputation</span> Medical procedure that removes a part of the body

Amputation is the removal of a limb by trauma, medical illness, or surgery. As a surgical measure, it is used to control pain or a disease process in the affected limb, such as malignancy or gangrene. In some cases, it is carried out on individuals as a preventive surgery for such problems. A special case is that of congenital amputation, a congenital disorder, where fetal limbs have been cut off by constrictive bands. In some countries, amputation is currently used to punish people who commit crimes. Amputation has also been used as a tactic in war and acts of terrorism; it may also occur as a war injury. In some cultures and religions, minor amputations or mutilations are considered a ritual accomplishment. When done by a person, the person executing the amputation is an amputator. The oldest evidence of this practice comes from a skeleton found buried in Liang Tebo cave, East Kalimantan, Indonesian Borneo dating back to at least 31,000 years ago, where it was done when the amputee was a young child.

<span class="mw-page-title-main">Gangrene</span> Type of tissue death by a lack of blood supply

Gangrene is a type of tissue death caused by a lack of blood supply. Symptoms may include a change in skin color to red or black, numbness, swelling, pain, skin breakdown, and coolness. The feet and hands are most commonly affected. If the gangrene is caused by an infectious agent, it may present with a fever or sepsis.

<span class="mw-page-title-main">Interventional radiology</span> Medical subspecialty

Interventional radiology (IR) is a medical specialty that performs various minimally-invasive procedures using medical imaging guidance, such as x-ray fluoroscopy, computed tomography, magnetic resonance imaging, or ultrasound. IR performs both diagnostic and therapeutic procedures through very small incisions or body orifices. Diagnostic IR procedures are those intended to help make a diagnosis or guide further medical treatment, and include image-guided biopsy of a tumor or injection of an imaging contrast agent into a hollow structure, such as a blood vessel or a duct. By contrast, therapeutic IR procedures provide direct treatment—they include catheter-based medicine delivery, medical device placement, and angioplasty of narrowed structures.

<span class="mw-page-title-main">Peripheral artery disease</span> Medical condition

Peripheral artery disease (PAD) is an abnormal narrowing of arteries other than those that supply the heart or brain. PAD can happen in any blood vessel, but it is more common in the legs than the arms.

<span class="mw-page-title-main">Ischemia</span> Restriction in blood supply to tissues

Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism. Ischemia is generally caused by problems with blood vessels, with resultant damage to or dysfunction of tissue i.e. hypoxia and microvascular dysfunction. It also implies local hypoxia in a part of a body resulting from constriction. Ischemia causes not only insufficiency of oxygen, but also reduced availability of nutrients and inadequate removal of metabolic wastes. Ischemia can be partial or total blockage. The inadequate delivery of oxygenated blood to the organs must be resolved either by treating the cause of the inadequate delivery or reducing the oxygen demand of the system that needs it. For example, patients with myocardial ischemia have a decreased blood flow to the heart and are prescribed with medications that reduce chronotrophy and ionotrophy to meet the new level of blood delivery supplied by the stenosed vasculature so that it is adequate.

Phlebitis is inflammation of a vein, usually in the legs. It most commonly occurs in superficial veins. Phlebitis often occurs in conjunction with thrombosis and is then called thrombophlebitis or superficial thrombophlebitis. Unlike deep vein thrombosis, the probability that superficial thrombophlebitis will cause a clot to break up and be transported in pieces to the lung is very low.

Intermittent claudication, also known as vascular claudication, is a symptom that describes muscle pain on mild exertion, classically in the calf muscle, which occurs during exercise, such as walking, and is relieved by a short period of rest. It is classically associated with early-stage peripheral artery disease, and can progress to critical limb ischemia unless treated or risk factors are modified and maintained.

<span class="mw-page-title-main">Fibromuscular dysplasia</span> Human arterial disease

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory disease of the blood vessels that causes abnormal growth within the wall of an artery. FMD has been found in nearly every arterial bed in the body, although the most commonly affected are the renal and carotid arteries.

<span class="mw-page-title-main">Vascular disease</span> Medical condition

Vascular disease is a class of diseases of the vessels of the circulatory system in the body, including blood vessels – the arteries and veins, and the lymphatic vessels. Vascular disease is a subgroup of cardiovascular disease. Disorders in this vast network of blood and lymph vessels can cause a range of health problems that can sometimes become severe, and fatal. Coronary heart disease for example, is the leading cause of death for men and women in the United States.

Ancrod is a defibrinogenating agent derived from the venom of the Malayan pit viper. Defibrinogenating blood produces an anticoagulant effect. Ancrod is not approved or marketed in any country. It is a thrombin-like serine protease.

Leo Buerger was an Austrian American pathologist, surgeon and urologist. Buerger's disease is named for him.

<span class="mw-page-title-main">Chronic venous insufficiency</span> Medical condition

Chronic venous insufficiency (CVI) is a medical condition in which blood pools in the veins, straining the walls of the vein. The most common cause of CVI is superficial venous reflux which is a treatable condition. As functional venous valves are required to provide for efficient blood return from the lower extremities, this condition typically affects the legs. If the impaired vein function causes significant symptoms, such as swelling and ulcer formation, it is referred to as chronic venous disease. It is sometimes called chronic peripheral venous insufficiency and should not be confused with post-thrombotic syndrome in which the deep veins have been damaged by previous deep vein thrombosis.

Felix von Winiwarter was an Austrian physician who was a native of Vienna.

<span class="mw-page-title-main">Cholesterol embolism</span> Medical condition

Cholesterol embolism occurs when cholesterol is released, usually from an atherosclerotic plaque, and travels as an embolus in the bloodstream to lodge causing an obstruction in blood vessels further away. Most commonly this causes skin symptoms, gangrene of the extremities and sometimes kidney failure; problems with other organs may arise, depending on the site at which the cholesterol crystals enter the bloodstream. When the kidneys are involved, the disease is referred to as atheroembolic renal disease. The diagnosis usually involves biopsy from an affected organ. Cholesterol embolism is treated by removing the cause and giving supportive therapy; statin drugs have been found to improve the prognosis.

<span class="mw-page-title-main">Arterial insufficiency ulcer</span> Medical condition

Arterial insufficiency ulcers are mostly located on the lateral surface of the ankle or the distal digits. They are commonly caused by peripheral artery disease (PAD).

<span class="mw-page-title-main">Acute limb ischaemia</span> Occurs when there is a sudden lack of blood flow to a limb

Acute limb ischaemia (ALI) occurs when there is a sudden lack of blood flow to a limb, within 14 days of symptoms onset. It is different from another condition which is more chronic called critical limb ischemia (CLD). CLD is the end stage of peripheral vascular disease where there is still some collateral circulation (alternate circulation pathways} that bring some blood to the distal parts of the limbs. While limbs in both acute and chronic limb ischemia may be pulseless, a chronically ischemic limb is typically warm and pink due to a well-developed collateral artery network and does not need emergency intervention to avoid limb loss.

Chronic limb threatening ischemia (CLTI), also known as critical limb ischemia (CLI), is an advanced stage of peripheral artery disease (PAD). It is defined as ischemic rest pain, arterial insufficiency ulcers, and gangrene. The latter two conditions are jointly referred to as tissue loss, reflecting the development of surface damage to the limb tissue due to the most severe stage of ischemia. Compared to the other manifestation of PAD, intermittent claudication, CLI has a negative prognosis within a year after the initial diagnosis, with 1-year amputation rates of approximately 12% and mortality of 50% at 5 years and 70% at 10 years.

<span class="mw-page-title-main">Femoropopliteal bypass</span> Leg artery surgical procedure

Popliteal bypass surgery, more commonly known as femoropopliteal bypass or more generally as lower extremity bypass surgery, is a surgical procedure used to treat diseased leg arteries above or below the knee. It is used as a medical intervention to salvage limbs that are at risk of amputation and to improve walking ability in people with severe intermittent claudication and ischemic rest pain.

<span class="mw-page-title-main">Diabetic foot infection</span> Medical condition

Diabetic foot infection is any infection of the foot in a diabetic person. The most frequent cause of hospitalization for diabetic patients is due to foot infections. Symptoms may include pus from a wound, redness, swelling, pain, warmth, tachycardia, or tachypnea. Complications can include infection of the bone, tissue death, amputation, or sepsis. They are common and occur equally frequently in males and females. Older people are more commonly affected.

<span class="mw-page-title-main">Arterial occlusion</span>

Arterial occlusion is a condition involving partial or complete blockage of blood flow through an artery. Arteries are blood vessels that carry oxygenated blood to body tissues. An occlusion of arteries disrupts oxygen and blood supply to tissues, leading to ischemia. Depending on the extent of ischemia, symptoms of arterial occlusion range from simple soreness and pain that can be relieved with rest, to a lack of sensation or paralysis that could require amputation.


  1. 1 2 3 4 5 6 Ferri FF (2003). Ferri's Clinical Advisor 2004: Instant Diagnosis and Treatment (6th ed.). p. 840. ISBN   978-0323026680.
  2. Joyce JW (May 1990). "Buerger's disease (thromboangiitis obliterans)". Rheumatic Disease Clinics of North America. 16 (2): 463–70. doi:10.1016/S0889-857X(21)01071-1. PMID   2189162.
  3. Mayo Clinic Staff. "Overview of Buerger's disease". Mayo Clinic. Retrieved 13 February 2016.
  4. "Thromboangiitis obliterans". Medline Plus. U.S. National Library of Medicine. Retrieved 13 February 2016.
  5. Porth C (2007). Essentials of Pathophysiology: Concepts of Altered Health States (2nd ed.). Lippincott Williams&Wilkins. p.  264. ISBN   9780781770873.
  6. Tanaka K (October 1998). "Pathology and pathogenesis of Buerger's disease". International Journal of Cardiology. 66 Suppl 1: S237-42. doi:10.1016/s0167-5273(98)00174-0. PMID   9951825.
  7. Fazeli B, Ravari H, Farzadnia M (December 2012). "Does a species of Rickettsia play a role in the pathophysiology of Buerger's disease?". Vascular. 20 (6): 334–6. doi:10.1258/vasc.2011.cr0271. PMID   21803838. S2CID   22660338.
  8. Olin JW (September 2000). "Thromboangiitis obliterans (Buerger's disease)". The New England Journal of Medicine. 343 (12): 864–9. doi:10.1056/NEJM200009213431207. PMID   10995867.
  9. Atlas of Clinical Diagnosis (2nd ed.). Elsevier Health Sciences. 2003. p. 238. ISBN   9780702026683.
  10. Matsudaira K, Seichi A, Kunogi J, Yamazaki T, Kobayashi A, Anamizu Y, et al. (January 2009). "The efficacy of prostaglandin E1 derivative in patients with lumbar spinal stenosis". Spine. 34 (2): 115–20. doi:10.1097/BRS.0b013e31818f924d. PMID   19112336. S2CID   22190177.
  11. 1 2 Cacione, Daniel G.; Macedo, Cristiane R.; do Carmo Novaes, Frederico; Baptista-Silva, Jose Cc (4 May 2020). "Pharmacological treatment for Buerger's disease". The Cochrane Database of Systematic Reviews. 5 (5): CD011033. doi:10.1002/14651858.CD011033.pub4. ISSN   1469-493X. PMC   7197514 . PMID   32364620.
  12. Clinical Surgery (2nd ed.). John Wiley & Sons. 2012. ISBN   978111834395-1.
  13. Cacione DG, do Carmo Novaes F, Moreno DH (October 2018). Cochrane Vascular Group (ed.). "Stem cell therapy for treatment of thromboangiitis obliterans (Buerger's disease)". The Cochrane Database of Systematic Reviews. 2018 (10): CD012794. doi:10.1002/14651858.CD012794.pub2. PMC   6516882 . PMID   30378681.
  14. Hussein EA, el Dorri A (1993). "Intra-arterial streptokinase as adjuvant therapy for complicated Buerger's disease: early trials". International Surgery. 78 (1): 54–8. PMID   8473086.
  15. 1 2 Piazza G, Creager MA (April 2010). "Thromboangiitis obliterans". Circulation. 121 (16): 1858–61. doi:10.1161/CIRCULATIONAHA.110.942383. PMC   2880529 . PMID   20421527.
  16. v. Winiwarter F (1879). "Ueber eine eigenthümliche Form von Endarteriitis und Endophlebitis mit Gangrän des Fusses". Archiv für Klinische Chirurgie. 23: 202–226.
  17. Buerger L (1908). "Thrombo-angiitis obliterans: a study of the vascular lesions leading to presenile spontaneous gangrene". American Journal of the Medical Sciences. 136: 567–580. doi:10.1097/00000441-190810000-00011. S2CID   31731903.
  18. McBeth J (2011). "Year of the Leg". Reporter: Forty Years Covering Asia. Singapore: Talisman Publishing. pp. 254–264. ISBN   9789810873646.
  19. Frialde M (December 10, 2015). "Duterte: I may not last 6 years in office". The Philippine Star. Retrieved December 17, 2015.

Further reading