Thromboangiitis obliterans

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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 /bɜːrɡər/ ; German pronunciation: [/byːɐ̯gəɐ̯/] ), 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]

Contents

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]

Pathophysiology

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.

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

Diagnosis

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 20–40 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.

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.

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).

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.

Prevention

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 ]

Treatment

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.

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] A 2020 Cochrane review found moderate certainty evidence that intravenous iloprost (prostacyclin analogue) is more effective than aspirin for relieving rest pain and healing ischemic ulcers and that there is no difference 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. Above-knee and below-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.

Prognosis

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.

Epidemiology

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]

History

Buerger's disease was first reported 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. [17] Buerger called it "presenile spontaneous gangrene" after studying amputations in 11 patients.

Notable sufferers

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.

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

Gangrene

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.

Interventional radiology

Interventional radiology (IR) is a medical subspecialty 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.

Peripheral artery disease

Peripheral artery disease (PAD) is an abnormal narrowing of arteries other than those that supply the heart or brain. When narrowing occurs in the heart, it is called coronary artery disease, and in the brain, it is called cerebrovascular disease. Peripheral artery disease most commonly affects the legs, but other arteries may also be involved – such as those of the arms, neck, or kidneys. The classic symptom is leg pain when walking which resolves with rest, known as intermittent claudication. Other symptoms include skin ulcers, bluish skin, cold skin, or abnormal nail and hair growth in the affected leg. Complications may include an infection or tissue death which may require amputation; coronary artery disease, or stroke. Up to 50% of people with PAD do not have symptoms.

Ischemia Restriction in blood supply to tissues

Ischemia or ischaemia is a restriction in blood supply to tissues, 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. It also means local anemia in a given part of a body sometimes resulting from constriction. Ischemia comprises not only insufficiency of oxygen, but also reduced availability of nutrients and inadequate removal of metabolic wastes. Ischemia can be partial or total.

Raynaud syndrome Medical condition in which spasm of arteries cause episodes of reduced blood flow

Raynaud syndrome, also known as Raynaud's phenomenon, is a medical condition in which spasm of arteries cause episodes of reduced blood flow. Typically, the fingers, and less commonly the toes, are involved. Rarely, the nose, ears, or lips are affected. The episodes result in the affected part turning white and then blue. Often, numbness or pain occurs. As blood flow returns, the area turns red and burns. The episodes typically last minutes, but can last several hours.

Vasculitis

Vasculitis is a group of disorders that destroy blood vessels by inflammation. Both arteries and veins are affected. Lymphangitis is sometimes considered a type of vasculitis. Vasculitis is primarily caused by leukocyte migration and resultant damage.

Phlebitis or venitis is the 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.

Polyarteritis nodosa

Polyarteritis nodosa (PAN), is a systemic necrotizing inflammation of blood vessels (vasculitis) affecting medium-sized muscular arteries, typically involving the arteries of the kidneys and other internal organs but generally sparing the lungs' circulation. Polyarteritis nodosa may be present in infants. In polyarteritis nodosa, small aneurysms are strung like the beads of a rosary, therefore making "rosary sign" an important diagnostic feature of the vasculitis. PAN is sometimes associated with infection by the hepatitis B or hepatitis C virus.

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.

Carotid artery stenosis

Carotid artery stenosis is a narrowing or constriction of any part of the carotid arteries, usually caused by atherosclerosis.

Fibromuscular dysplasia

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 common arteries affected are the renal and carotid arteries.

Vascular disease

Vascular disease is a class of diseases of the blood vessels – the arteries and veins of the circulatory system of the body. It is a subgroup of cardiovascular disease. Disorders in this vast network of blood vessels, can cause a range of health problems which can be severe or prove fatal.

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.

Nicotinyl alcohol

Nicotinyl alcohol (pyridylcarbinol) is a niacin derivative used as a hypolipidemic agent and as a vasodilator. It causes flushing and may decrease blood pressure.

Cryofibrinogenemia refers to a condition classified as a fibrinogen disorder in which the chilling of an individual's blood plasma from the normal body temperature of 37 °C to the near-freezing temperature of 4 °C causes the reversible precipitation of a complex containing fibrinogen, fibrin, fibronectin, and, occasionally, small amounts of fibrin split products, albumin, immunoglobulins and other plasma proteins. Returning this plasma to 37 °C resolubilizes the precipitate.

Arterial insufficiency ulcer

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).

Acute limb ischaemia 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.

Blood clots are a relatively common occurrence in the general population and are seen in approximately 1-2% of the population by age 60. Typically blood clots develop in the deep veins of the lower extremities, deep vein thrombosis (DVT) or as a blood clot in the lung, pulmonary embolism (PE). A very small number of people who develop blood clots have a more serious and often life-threatening condition, known as Thrombotic Storm (TS). TS is characterized by the development of more than one blood clot in a short period of time. These clots often occur in multiple and sometimes unusual locations in the body and are often difficult to treat. TS may be associated with an existing condition or situation that predisposes a person to blood clots such as injury, infection, or pregnancy. In many cases a risk assessment will identify interventions that will prevent the formation of blood clots.

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.

Blood vessel disorder generally refers to the narrowing, hardening or enlargement of arteries and veins. It is often due to the build-up of fatty deposits in the lumen of blood vessels or infection of the vessel wall. This can occur in various locations such as coronary blood vessels, peripheral arteries and veins. The narrowed arteries would block the blood supply to different organs and tissues. In severe conditions, it may develop into more critical health problems like myocardial infarction, stroke or heart failure, which are some of the major reasons of death.

References

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  2. Joyce JW (May 1990). "Buerger's disease (thromboangiitis obliterans)". Rheumatic Diseases Clinics of North America. 16 (2): 463–70. 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.
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  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.
  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.
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  11. 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: 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.
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  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. von Winiwarter F (1879). "Ueber eine eigenthumliche Form von Endarteriitis und Endophlebitis mit Gangran des Fusses". Arch Klin Chir. 23: 202–26.
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  18. McBeth 2011, pp. 254-264.
  19. Frialde M (December 10, 2015). "Duterte: I may not last 6 years in office". The Philippine Star. Retrieved December 17, 2015.

Further reading

Classification
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External resources