Buerger's disease - AbsoluteAstronomy.com

Not to be confused with Berger's disease (IgA nephropathy
IgA nephropathy
IgA nephropathy is a form of glomerulonephritis...

)

Thromboangiitis obliterans (also known as Buerger's disease) is a recurring progressive inflammation

Inflammation

Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...

and thrombosis

Thrombosis

Thrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot to prevent blood loss...

(clotting) of small and medium arteries

Artery

Arteries are blood vessels that carry blood away from the heart. This blood is normally oxygenated, exceptions made for the pulmonary and umbilical arteries....

and vein

Vein

In the circulatory system, veins are blood vessels that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated blood to the heart...

s of the hands and feet. It is strongly associated with use of tobacco

Tobacco

Tobacco is an agricultural product processed from the leaves of plants in the genus Nicotiana. It can be consumed, used as a pesticide and, in the form of nicotine tartrate, used in some medicines...

products, primarily from smoking

Tobacco smoking

Tobacco smoking is the practice where tobacco is burned and the resulting smoke is inhaled. The practice may have begun as early as 5000–3000 BCE. Tobacco was introduced to Eurasia in the late 16th century where it followed common trade routes...

, but also from smokeless tobacco

Smokeless tobacco

Smokeless Tobacco may refer to:* Dipping tobacco, a type of tobacco that is placed between the lower or upper lip and gums.* Chewing tobacco, a type of tobacco that is chewed.* Snuff, a type of tobacco that is insufflated or "snuffed" through the nose....

Signs and symptoms

There is a recurrent acute and chronic inflammation

Inflammation

and thrombosis

Thrombosis

of arteries

Artery

Arteries are blood vessels that carry blood away from the heart. This blood is normally oxygenated, exceptions made for the pulmonary and umbilical arteries....

and vein

Vein

s of the hands and feet. The main symptom is pain

Pain

Pain is an unpleasant sensation often caused by intense or damaging stimuli such as stubbing a toe, burning a finger, putting iodine on a cut, and bumping the "funny bone."...

in the affected areas. Ulcerations and gangrene

Gangrene

Gangrene is a serious and potentially life-threatening condition that arises when a considerable mass of body tissue dies . This may occur after an injury or infection, or in people suffering from any chronic health problem affecting blood circulation. The primary cause of gangrene is reduced blood...

in the extremities are common complication

Complication (medicine)

Complication, in medicine, is an unfavorable evolution of a disease, a health condition or a medical treatment. The disease can become worse in its severity or show a higher number of signs, symptoms or new pathological changes, become widespread throughout the body or affect other organ systems. A...

s, often resulting in the need for amputation

Amputation

Amputation is the removal of a body extremity by trauma, prolonged constriction, 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 preventative surgery for...

of the involved extremity.

This disease was first reported by Leo Buerger

Leo Buerger

Leo Buerger was an Austrian pathologist, surgeon and urologist. Buerger's disease is named for him.- Family and education :...

in 1908, who described a disease in which the characteristic pathologic findings — acute inflammation and thrombosis (clotting) of arteries and veins — affected the hands and feet. Another name for Buerger’s disease is thromboangiitis obliterans.

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:

Typically between 20–40 years old and male, although recently females have been diagnosed.
Current (or recent) history of tobacco use
Presence of distal extremity ischemia
Ischemia
In medicine, ischemia is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. It may also be spelled ischaemia or ischæmia...

(indicated by claudication
Claudication
Claudication, literally 'limping' , is a medical term usually referring to impairment in walking, or pain, discomfort or tiredness in the legs that occurs during walking and is relieved by rest. The perceived level of pain from claudication can be mild to extremely severe. Claudication is most...

, pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as ultrasound
Exclusion of other autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.
Exclusion of a proximal source of emboli by echocardiography and arteriography
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 Buerger’s there is no treatment known to be effective.

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 of the blood, and others.

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. Angiograms may also show occlusions (blockages) or stenosis (narrowings) in multiple areas of both the arms and legs. The changes are particularly apparent in the blood vessels in the lower right hand portion of the picture (the ulnar artery distribution).

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.

Pathophysiology

There are characteristic pathologic

Pathology

Pathology is the precise study and diagnosis of disease. The word pathology is from Ancient Greek , pathos, "feeling, suffering"; and , -logia, "the study of". Pathologization, to pathologize, refers to the process of defining a condition or behavior as pathological, e.g. pathological gambling....

findings of acute inflammation

Inflammation

and thrombosis

Thrombosis

(clotting) of arteries

Artery

Arteries are blood vessels that carry blood away from the heart. This blood is normally oxygenated, exceptions made for the pulmonary and umbilical arteries....

and vein

Vein

s of the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still largely unknown. It is suspected that immunological

Immunology

Immunology is a broad branch of biomedical science that covers the study of all aspects of the immune system in all organisms. It deals with the physiological functioning of the immune system in states of both health and diseases; malfunctions of the immune system in immunological disorders ; the...

reactions play a role.

Most patients with Buerger’s are regular smokers, but some cases occur in patients who only smoke “moderately”; others have been reported in users of smokeless tobacco. It has been postulated that Buerger’s disease is an “autoimmune” reaction (one in which the body’s immune system attacks the body’s own tissues) triggered by some constituent of tobacco.

Prevention

The cause of the disease is unknown but thought to be autoimmune in nature and heavily linked to tobacco use in patients with Buerger's as primary disease. There have also been links to persons with digestive disorders.

A possible role for Rickettsia
Rickettsia
Rickettsia is a genus of non-motile, Gram-negative, non-sporeforming, highly pleomorphic bacteria that can present as cocci , rods or thread-like . Being obligate intracellular parasites, the Rickettsia survival depends on entry, growth, and replication within the cytoplasm of eukaryotic host cells...

in this disease has been proposed.

Treatment

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

Vascular surgery

Vascular surgery

Vascular surgery is a specialty of surgery in which diseases of the vascular system, or arteries and veins, are managed by medical therapy, minimally-invasive catheter procedures, and surgical reconstruction. The specialty evolved from general and cardiac surgery...

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.

Streptokinase

Streptokinase

Streptokinase , a protein secreted by several species of streptococci can bind and activate human plasminogen. SK is used as an effective and inexpensive thrombolysis medication in some cases of myocardial infarction and pulmonary embolism...

has been proposed as adjuvant therapy in some cases.

In addition Limaprost, an oral PGE1 derivative, has shown efficacy in one published randomized trial.

Prognosis

Buerger's is not immediately fatal, but it is life-shortening. Amputation is common and major amputations (of limbs rather than fingers/toes) are almost twice as common in patients who continue to smoke. Death rate has not been consistently shown as higher in patients who do not cease smoking but for this and other health concerns quitting is highly recommended. Female patients tend to show much higher longevity rates than men.

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 (thinning of the blood with aspirin or other agents to prevent clots) have not proven effective. The only 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. It is more common in , Japan

Japan

Japan is an island nation in East Asia. Located in the Pacific Ocean, it lies to the east of the Sea of Japan, China, North Korea, South Korea and Russia, stretching from the Sea of Okhotsk in the north to the East China Sea and Taiwan in the south...

, India

India

India , officially the Republic of India , is a country in South Asia. It is the seventh-largest country by geographical area, the second-most populous country with over 1.2 billion people, and the most populous democracy in the world...

, and Manipur

Manipur

Manipur is a state in northeastern India, with the city of Imphal as its capital. Manipur is bounded by the Indian states of Nagaland to the north, Mizoram to the south and Assam to the west; it also borders Burma to the east. It covers an area of...

along the "old silk route" than in the United States

United States

The United States of America is a federal constitutional republic comprising fifty states and a federal district...

and Europe

Europe

Europe is, by convention, one of the world's seven continents. Comprising the westernmost peninsula of Eurasia, Europe is generally 'divided' from Asia to its east by the watershed divides of the Ural and Caucasus Mountains, the Ural River, the Caspian and Black Seas, and the waterways connecting...

. The disease is most common among South Asians. Incidence of Thromboangiitis obliterans is 8 to 12 per 100,000 adults in the United States

United States

The United States of America is a federal constitutional republic comprising fifty states and a federal district...

(0.75% of all patients with peripheral arterial disease).

History

Buerger's disease was first reported by Felix von Winiwarter

Felix von Winiwarter

Felix von Winiwarter was an Austrian physician who was a native of Vienna. In 1876 he earned his doctorate at the University of Vienna, and remained in Vienna as an assistant in the clinic of Heinrich von Bamberger...

in 1879 in Austria

Austria

Austria , officially the Republic of Austria , is a landlocked country of roughly 8.4 million people in Central Europe. It is bordered by the Czech Republic and Germany to the north, Slovakia and Hungary to the east, Slovenia and Italy to the south, and Switzerland and Liechtenstein to the...

. It wasn't until 1908, however, that the disease was given its first accurate pathological description, by Leo Buerger

Leo Buerger

Leo Buerger was an Austrian pathologist, surgeon and urologist. Buerger's disease is named for him.- Family and education :...

at Mount Sinai Hospital

Mount Sinai Hospital, New York

Mount Sinai Hospital, founded in 1852, is one of the oldest and largest teaching hospitals in the United States. In 2011-2012, Mount Sinai Hospital was ranked as one of America's best hospitals by U.S...

in New York City

New York City

New York is the most populous city in the United States and the center of the New York Metropolitan Area, one of the most populous metropolitan areas in the world. New York exerts a significant impact upon global commerce, finance, media, art, fashion, research, technology, education, and...

. Buerger called it "presenile spontaneous gangrene" after studying amputations in 11 patients.

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 in late 1948 and early 1949. 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 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 March 12, 1949, the King underwent a lumbar sympathectomy

Sympathectomy

A Sympathectomy is a procedure during which at least one sympathetic ganglion is removed.An example is endoscopic thoracic sympathectomy....

, performed at Buckingham Palace

Buckingham Palace

Buckingham Palace, in London, is the principal residence and office of the British monarch. Located in the City of Westminster, the palace is a setting for state occasions and royal hospitality...

by Dr. James R. Learmonth. The operation, as such, was successful, but the King was warned that it was a pallative, 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.

External links

Brandon Carmichael
Johns Hopkins University http://vasculitis.med.jhu.edu/typesof/buergers.html

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