irst Description

This disease was first reported by Buerger 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 Buergers Disease is thromboangiitis
obliterans.

Who gets Buergers Disease (the typical patient)?

The classic Buergers Disease patient is a young male (e.g., 2040 years old) who is a heavy
cigarette smoker. More recently, however, a higher percentage of women and people over the
age of 50 have been recognized to have this disease. Buergers disease is most common in the
Orient, Southeast Asia, India and the Middle East, but appears to be rare among African
Americans.

Classic symptoms and signs of Buergers Disease

The initial symptoms of Buergers Disease often include claudication (pain induced by
insufficient blood flow during exercise) in the feet and/or hands, or pain in these areas at rest.
The pain typically begins in the extremities but may radiate to other (more central) parts of the
body. Other signs and symptoms of this disease may include numbness and/or tingling in the
limbs and Raynauds phenomenon (a condition in which the distal extremities fingers, toes,
hands, feet turn white upon exposure to cold). Skin ulcerations and gangrene (pictured
below) of the digits (fingers and toes) are common in Buergers disease. Pain may be very
intense in the affected regions.

An angiogram demonstrating lack of blood flow to vessels of the hand (figure below). This
decreased blood flow (ischemia) led to ulcers of the fingers and severe pain.
An abnormal result from an angiogram of the hand (figure below).

Despite the severity of ischemia (lack of blood flow) to the distal extremities that occurs in
Buergers, the disease does not involve other organs, unlike many other forms of vasculitis. Even
as ulcers and gangrene develop in the digits, organs such as the lung, kidneys, brain, and
gastrointestinal (GI) tract remain unaffected. The reasons for the confinement to the extremities
and sparing of other organs are not known.

What Causes Buergers Disease?

The association of Buergers Disease with tobacco use, particularly cigarette smoking, cannot be
overemphasized. Most patients with Buergers are heavy smokers, but some cases occur in
patients who smoke moderately; others have been reported in users of smokeless tobacco. It
has been postulated that Buergers Disease is an autoimmune reaction (one in which the
bodys immune system attacks the bodys own tissues) triggered by some constituent of tobacco.

Pictured below, are a patients fingertips that have developed gangrene. This is a very painful
condition which sometimes requires amputation of the affected area.
How is Buergers diagnosed?
Buergers 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 Buergers Disease (for
Buergers, there is only one treatment known to be effective: complete smoking cessation see
below).

Diseases with which Buergers Disease may be confused include atherosclerosis (buildup of
cholesterol plaques in the arteries), endocarditis (an infection of the lining of the heart), other
types of vasculitis, severe Raynauds 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
Buergers disease. In the proper clinical setting, certain angiographic findings are diagnostic of
Buergers. 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 stenoses (narrowings) in multiple areas of both the arms and
legs.

Pictured below on the left is a normal angiogram. On the right, is an abnormal angiogram of an
arm demonstrating the classic corkscrew appearance of arteries to the hand. The changes are
particularly apparent in the blood vessels in the lower right hand portion of the picture (the ulnar
artery distribution).
In order to rule out other forms of vasculitis (by excluding involvement of vascular regions
atypical for Buergers), 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.

Treatment and Course of Buergers

It is essential that patients with Buergers disease stop smoking immediately and completely.
This is the only treatment known to be effective in Buergers disease. Patients who continue to
smoke are generally the ones who require amputation of fingers and toes.

Despite the clear presence of inflammation in this disorder, anti-inflammatory agents such as
steroids have not been shown to be beneficial. Similarly, strategies of anticoagulation (thinning
of the blood with aspirin or other agents to prevent clots) have not proven effective. The only
way to prevent the progression of the disease is to abstain from all tobacco products.

V
Etiology and Pathophysiology:

Smoking is very closely related to Buerger's disease and smoking history is one of the criterion
for diagnosing the disease. In general if the patient absolutely abandons smoking the course of
the disease will be invariably benign, but if smoking continues any treatment will ultimately be
futile. Though "passive smoking" has adverse effect on cardiovascular system, non smokers
should never develop the disease. Active smokers can be indentified by measuring levels of
continine, the major metabolite of nicotine in urine. Since all smokers do not develop the disease
an immunopathogenesis is considered probable. It has been proposed in Japanese that presence
of a gene linked to some HLA antigens might control the susceptibility to the disease.

Socioeconomic conditions, work environment may also play in etiology as the disease is seen
more in out door and manual workers. Hypercoagulable state has been observed in association
with the disease. Hepatitis B Virus and rickettssiosis may contribute to pathogenesis, but this role
is uncertain.

Buergers disease is an inflammatry occlusive disease which involves all layers of medium sized
and small arteries of the extremitiles. Involved superficial veins bear a close resemblance to
those in the affected artery. Majority of the patients develop critical limb ischemia with trophic
lesions are distal to ankle, the anklebrachial doppler index could be normal in early stage. Toe
pressures can be measured and if it is less than 30 mm Hg, the healing of ulcers is unlikely. The
disease though commences peripherally, may gradually extend proximately occluding the larger
arteries.

Clinical Presentation : varies with the stage of the disease. The patients may present with foot
claudication and later with calf claudication. Gangrene and ulceration may follow the above
symptoms, but many times they may occur without previous history of claudication. The
stepwise progession of the disease as seen in atherosclerasis may not occur in Buergers disease.
Parasthesia, coldness and skin colour changes are common complaints. Dependent rubor and
slow venous filling are commonly seen. Gangrene and ulceration usually follow minor trauma
and with development of secondary infection they may progress proximally and are associated
with intolerable rest pain.

Recurrent superficial thrombophlebitis marked by redness and tenderness over the affected vain,
can occur in arm, leg or the foot. The symptoms usually disappear over 2-3 weeks, leaving
behind blackish-brown pigmentation. "Phlebitis migrans" is characteristic of Buergers disease,
but is often missed both by the patient and the doctor.

Of 255 patients treated by shionaya from an institution in India 98% were males. The major
presenting symptoms were.

1. Parasthesia, Coldness, Cyanosis - 37%

2. Gangrene or Ulcer - 18%
3. Foot Claudication - 15%
4. Calf Claudication - 16%
5. Rest pain - 10%
 6. Thrombophlebitis - 3%

On continuous follow up-72% develop Ulcer/Gangrene, 42% develop phlebitis migrans and
about 90% eventually have upper extremity involvement. In the above series 83% had 3 or 4
limb involvement and 17% had 2 limb involvement and NONE had a single limb involvement.

In our experience with about 80 patients with Buergers disease, we have not seen a single patient
without ulcer or gangrene!

The criteria for diagnosis of Burgers disease include (1) History of smoking (2) Onset before the
age of 50 years (3) Infrapopliteal arterial occlusive disease (4) Either upper limb involvement or
phlebitis migrans (5) Absence of atherosclerotic risk factors other than smoking. Arteriographic
findings serve as supporting evidence and will be discussed later.

Diagnostic Studies:

Though history and physical exam are sufficient to diagnose Buerger's disease, diagnostic studies
are required for objective evalution and to select appropriate therapy.

Duplex Scanning : is useful in initial evaluation of the patient. Since these patient may have
multiple "skip" lesions and also have significant distal disease, arteriogram is a must before any
interventional therapy is planned.

ARTERIOGRAM remains the "gold standard" for evaluation of arterial occlusive disease.
Digital subtraction angiography is better in evaluation of distal occlusions. As mentioned above
multisegmental occlusion of distal limb arteries are characteratic of Buergers disease. There is
usually an extensive network of collaterals and these have a "corkscrew" or "root like
oppearance".

About 60% of patents have occulusion of infrapopliteal arteries, about 32% have involvement of
femoral popliteal segment and about 8% have aortoiliac disease. Arterial thromboses can extend
proximally in aortic lesions causing infrarenal occlusion of the aorta. Involvement of other
arteries like visceral vessels, coronary arteries are extremely rare and hence these patients have a
normal life span.

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. Ulcerations and gangrene in the
extremities are common complications, often resulting in the need for amputation of the involved
extremity.

This disease was first reported by Leo Buerger 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 Buergers disease is thromboangiitis
obliterans.

Causes and incidence

Buergers disease is caused by vasculitis, an inflammation of blood vessels, primarily of the
hands and feet. The vessels become constricted or totally blocked, reducing blood flow to the
tissues and resulting in pain and, eventually, damage.

This disorder occurs in 6 of every 10,000 people. Incidence is highest among males ages 20 to 40
who have a history of smoking or chewing tobacco. It may be associated with a history of
Raynauds disease and may occur in people with autoimmune disease.

Signs and symptoms

Buergers disease typically produces intermittent claudication of the instep, which is
aggravated by exercise and relieved by rest. During exposure to low temperature, the feet
initially become cold, cyanotic, and numb; later, they redden, become hot, and tingle.
Occasionally, Buergers disease also affects the hands, possibly resulting in painful fingertip
ulcerations. Associated signs and symptoms may include impaired peripheral pulses, migratory
superficial thrombophlebitis and, in later stages, ulceration, muscle atrophy, and gangrene.

Diagnosis
Patient history and physical examination strongly suggest Buergers disease. Supportive
diagnostic tests include:

Doppler ultrasonography to show diminished circulation in the peripheral vessels

plethysmography to help detect decreased circulation in the peripheral vessels

angiography or arteriography to locate lesions and rule out atherosclerosis.

Treatment
The primary goals of treatment are to relieve symptoms and prevent complications. Such therapy
may include an exercise program that uses gravity to fill and drain the blood vessels or, in severe
disease, a lumbar sympathectomy to increase blood supply to the skin. Aspirin and vasodilators
may also be used. Amputation may be necessary for nonhealing ulcers, intractable pain, or
gangrene.

Special considerations
Strongly urge the patient to stop smoking to enhance the treatments effectiveness. Symptoms
may disappear if he stops his tobacco use. If necessary, refer him to a self-help group to stop
smoking.

Warn the patient to avoid precipitating factors, such as emotional stress, exposure to extreme
temperatures, and trauma.

Teach the patient proper foot care, especially the importance of wearing well-fitting shoes and
cotton or wool socks. Show him how to inspect his feet daily for cuts, abrasions, and signs of
skin breakdown, such as redness and soreness. Remind him to seek medical attention at once
after any trauma.

If the patient has ulcers and gangrene, enforce bed rest and use a padded footboard or bed
cradle to prevent pressure from bed linens. Protect the feet with soft padding. Wash them gently
with a mild soap and tepid water, rinse thoroughly, and pat dry with a soft towel.

Provide emotional support. If necessary, refer the patient for psychological counseling to help
him cope with restrictions imposed by this chronic disease. If he has undergone amputation,
assess rehabilitative needs, especially regarding changes in body image. Refer him to physical
therapists, occupational therapists, and social service agencies, as needed.

Book Source Details

Book Title: Professional Guide to Diseases (Eighth Edition)
Author(s): Springhouse
Year of Publication: 2005
Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright 2005 Lippincott
Williams & Wilkins.

Read more at http://www.wrongdiagnosis.com/b/buergers_disease/book-diseases-

7b.htm?ktrack=kcplink

Buerger's Disease: Overview

Alternative Names: (Inflammatory) Occlusive Peripheral Vascular Disease or Thromboangiitis

Obliterans (TAO).

Buerger's disease is characterized by narrowing or blockage (occlusion) of the veins and arteries of the
extremities, resulting in reduced blood flow to these areas (peripheral vascular disease). The legs are
affected more often than the arms. (Article continues below...)
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Please note that it is extremely important to obtain an accurate diagnosis before trying to find a cure.
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Causes and Development

Buerger's disease occurs predominantly in men aged 20 to 40 who smoke cigarettes; only about 5% of
cases occur in women. Although the mechanism of the disease is unknown, the relationship of smoking
to its occurrence and progression is apparent.