Buerger's disease

Filed under: Heart & Vascular Buerger's disease (thromboangiitis obliterans) is a rare disease of the arteries and veins in the arms and legs. In Buerger's disease, your blood vessels become inflamed, swell and can become blocked with blood clots (thrombi). This eventually damages or destroys skin tissues and may lead to infection and gangrene. Buerger's disease usually first shows in the hands and feet and may eventually affect larger areas of your arms and legs.Buerger's disease is rare in the United States, but is more common in the Middle East and Far East. Buerger's disease usually affects men younger than 40 years of age, though it's becoming more common in women.

Virtually everyone diagnosed with Buerger's disease smokes cigarettes or uses other forms of tobacco, such as chewing tobacco. Quitting all forms of tobacco is the only way to stop Buerger's disease. For those who don't quit, amputation of all or part of a limb may be necessary.

Buerger's disease symptoms include:

Pain that may come and go in your legs and feet or your arms and hands. This pain typically occurs when you use your hands or feet and eases when you stop activity (claudication).

Inflammation along a vein just below the skin's surface (due to a blood clot in the vein). Fingers and toes that turn pale when exposed to cold (Raynaud's phenomenon). Painful open sores on your fingers and toes.

When to see a doctor See your doctor if you think you may have signs or symptoms of Buerger's disease.

CAUSES

It isn't clear what triggers Buerger's disease. It's possible that some people may have a genetic predisposition to the disease. The condition is characterized by swelling in the arteries and veins of the arms and legs. The cells that cause the inflammation and swelling — and eventually blood clots — form in the vessels leading to your hands and feet and block the blood flow to those parts of your body.

Reduced blood flow means that the skin tissue in your hands and feet doesn't get adequate oxygen and nutrients. This leads to the signs and symptoms of Buerger's disease, beginning with pain and weakness in your fingers and toes and spreading to other parts of your arms and legs.

RISK FACTORS

Tobacco use Cigarette smoking greatly increases your risk of Buerger's disease. Heavy cigarette smokers (people who smoke one and a half packs a day or more) are most likely to develop Buerger's disease, though it can occur in people who use any form of tobacco, including cigars and chewing tobacco. People who smoke hand-rolled cigarettes using raw tobacco may have the greatest risk of Buerger's disease.

It isn't clear how tobacco use increases your risk of Buerger's disease, but virtually everyone diagnosed with Buerger's disease uses tobacco. It's thought that chemicals in tobacco may irritate the lining of your blood vessels, causing them to swell. Rates of Buerger's disease are highest in areas of the Middle East and Far East where heavy smoking is most common.

Although secondhand smoke isn't thought to be a major risk factor for Buerger's disease, if you're diagnosed, you should stay away from people who are smoking. Secondhand smoke could worsen your condition.

Chronic gum disease Long-term infection of the gums is also associated with the development of Buerger's disease.

COMPLICATIONS

If Buerger's disease worsens, blood flow to your arms and legs decreases. This is due to blockages that make it hard for blood to reach the tips of your fingers and toes. Tissues that don't receive blood don't get the oxygen and nutrients they need to survive. This can cause the skin and tissue on the ends of your fingers and toes to die (gangrene). Signs and symptoms of gangrene include black or blue skin, a loss of feeling in the affected finger or toe, and a foul smell from the affected area. Gangrene is a serious condition that usually requires amputation of the affected finger or toe.\

Preparing for your appointment

You're likely to start by seeing your family doctor or primary care provider, who may eventually refer you to someone who specializes in blood vessel diseases (cardiologist).

What you can do To make the most of your appointment, come prepared with information and questions for your doctor.

Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.

Write down key personal information, including whether you've ever smoked, and how many packs a day, or if you've had repetitive trauma to your hands or feet, such as from using a jackhammer or other vibrating tools.

Make a list of all medications, as well as any vitamins or supplements, that you're taking. Take a family member or friend along, if possible. Sometimes it can be difficult to soak up all the information provided to

you during an appointment. Someone who accompanies you may remember something that you missed or forgot. Write down questions to ask your doctor.

Some basic questions to ask your doctor include:

What is likely causing my symptoms or condition? Other than the most likely cause, what are other possible causes for my symptoms or condition? What kinds of tests do I need? Is my condition likely temporary or chronic? What is the best course of action? What are the alternatives to the primary approach that you're suggesting? I have these other health conditions. How can I best manage them together? Are there any restrictions that I need to follow? Should I see a specialist? What will that cost, and will my insurance cover seeing a specialist? (You may need to check

with your insurance provider for this information.) Are there any brochures or other printed material that I can take home with me? What websites do you recommend

visiting?In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment.

What to expect from your doctor Your doctor may ask:

When did you begin experiencing symptoms? Have your symptoms been continuous or occasional? How severe are your symptoms? What, if anything, seems to improve your symptoms? What, if anything, appears to worsen your symptoms? Do you use tobacco in any form now or have you ever used it? Do your fingers change color in response to cold? Have you had repetitive trauma to the affected area from tools?

Tests and diagnosisWhile no tests can confirm whether you have Buerger's disease, your doctor will likely order tests to rule out other more common conditions or confirm suspicion of Buerger's disease brought on by your signs and symptoms. Tests may include:

Blood tests Blood tests to look for certain substances can rule out other conditions that may cause similar signs and symptoms. For instance, blood tests can help rule out scleroderma, lupus, blood-clotting disorders and diabetes, along with other diseases and conditions.

The Allen's test Your doctor may perform a simple test called the Allen's test to check blood flow through the arteries carrying blood to your hands. In the Allen's test, you make a tight fist, which forces the blood out of your hand. Your doctor presses on the arteries at each side of your wrist to slow the flow of blood back into your hand, making your hand lose its normal color. Next, you open your hand and your doctor releases the pressure on one artery, then the other. How quickly the color returns to your hand may give a general indication about the health of your arteries. Slow blood flow into your hand may indicate a problem, such as Buerger's disease.

Angiogram An angiogram, also called an arteriogram, helps doctors see the condition of your arteries. A special dye is injected into an artery, after which you undergo X-rays or other imaging tests. The dye helps to delineate any artery blockages that show up on the images. Your doctor may order angiograms of both your arms and your legs — even if you don't have signs and symptoms of Buerger's disease in all of your limbs. Buerger's disease almost always affects more than one limb, so even though you may not have signs and symptoms in your other limbs, this test may detect early signs of vessel damage.

Treatments and drugsSmoking cessation Although no treatment can cure Buerger's disease, the most effective way to halt the disease's progress is to quit using all tobacco products. Even a few cigarettes a day can worsen the disease.

Your doctor can counsel you and recommend medications to help you stop smoking and stop the swelling in your blood vessels. You'll need to avoid nicotine replacement products because they supply nicotine, which activates Buerger's disease; there are non-nicotine products that you can use. If the disease is still active, your doctor may check your urine for the presence of nicotine to see if you're still smoking.

Another option is a residential smoking-cessation program. In these programs, you stay at a treatment facility, sometimes a hospital, for a set number of days or weeks. During that time you participate in daily counseling sessions and other activities to help you deal with the cravings for cigarettes and to help you learn to live tobacco-free.

Other treatments Other treatment approaches exist but are less effective. Options include:

Medications to dilate blood vessels, improve blood flow or dissolve blood clots Intermittent compression of the arms and legs to increase blood flow to your extremities Spinal cord stimulation Surgery to cut the nerves to the affected area (surgical sympathectomy) to control pain and increase blood flow, although

this procedure is controversial Medications to stimulate growth of new blood vessels (therapeutic angiogenesis), an approach that is considered

experimental by many Amputation, if infection or gangrene occurs

Lifestyle and home remediesTake care of your fingers and toes if you have Buerger's disease. Check the skin on your arms and legs daily for cuts and scrapes, keeping in mind that if you've lost feeling to a finger or toe you may not feel, for example, a cut when it happens. Keep your fingers and toes protected and avoid exposing them to cold.

Low blood flow to your extremities means your body can't resist infection as easily. Small cuts and scrapes can easily turn into serious infections. Clean any cut with water, apply antibiotic ointment and cover it with a clean bandage. Keep an eye on any cuts or scrapes to make sure they're healing. If they get worse or heal slowly, see your doctor promptly.

Visit your dentist regularly to keep your gums and teeth in good health and avoid gum disease, which in its chronic form is associated with Buerger's disease.

PreventionQuit using tobacco in any form Virtually everyone who has Buerger's disease has used tobacco in some form, most prominently cigarettes. To prevent Buerger's disease, it's important to not use tobacco.

Quitting smoking can be hard. If you're like most people who smoke, you've probably tried to quit in the past, but haven't been successful. It's never too late to try again. Talk to your doctor about strategies to help you quit.

©1998-2013 Mayo Foundation for Medical Education and Research (MFMER). Terms of use. 

http://edition.cnn.com/HEALTH/library/buergers-disease/DS00807.html

Background

Buerger disease, a nonatherosclerotic vascular disease also known as thromboangiitis obliterans (TAO), is characterized by the absence or minimal presence of atheromas, segmental vascular inflammation, vasoocclusive phenomenon, and involvement of small- and medium-sized arteries and veins of the upper and lower extremities. The condition is strongly associated with heavy tobacco use, and progression of the disease is closely linked to continued use. The typical presentations are rest pain, unremitting ischemic ulcerations, and gangrene of the digits of hands and feet, and as the disease evolves, the patients may require several surgical amputations.[1, 2]

The first reported case of thromboangiitis obliterans was described in Germany by von Winiwarter in an 1879 article titled "A strange form of endarteritis and endophlebitis with gangrene of the feet."[3] A little more than a quarter of a century later, in Brookline, NY, Leo Buerger published a detailed description of the disease in which he referred to the clinical presentation of thromboangiitis obliterans as "presenile spontaneous gangrene."[4] The paper discussed the pathological findings in 11 limbs amputated from Jewish patients with the disease.

PathophysiologyWhile the etiology of Buerger disease is unknown, exposure to tobacco is essential for both initiation and progression of the disease. The notion that the condition is linked to tobacco exposure is supported by the fact that the disease is more common in countries with heavy use of tobacco and is perhaps most common among natives of Bangladesh who smoke a specific type of cigarettes, homemade from raw tobacco, called "bidi." While the overwhelming majority of patients with Buerger disease smoke, a few cases have been reported in nonsmokers that have been attributed to the use of chewing tobacco.

The disease mechanism underlying Buerger disease remains unclear, but a few observations have led investigators to implicate an immunologic phenomenon that leads to vasodysfunction and inflammatory thrombi. Patients with the disease show hypersensitivity to intradermally injected tobacco extracts, have increased cellular sensitivity to types I and III collagen, have elevated serum anti–endothelial cell antibody titers, and have impaired peripheral vasculature endothelium-dependent vasorelaxation. Increased prevalence of HLA-A9, HLA-A54, and HLA-B5 is observed in these patients, which suggests a genetic component to the 

EpidemiologyFrequency

United StatesThe prevalence of Buerger disease has decreased over the past half decade, partly because the prevalence of smoking has decreased, but also because the diagnostic criteria have become more stringent. In 1947, the prevalence of the disease in the United States was 104 cases per 100,000 population. More recently, prevalence has been estimated at 12.6-20 cases per 100,000 population.

Mortality/Morbidity

Death from Buerger disease is rare, but in patients with the disease who continue to smoke, 43% require 1 or more amputations in 7.6 years. Most recently, in a December 2004 CDC publication, the 2002 deaths report in the United States divided by cause of death, month, race, and sex (based on the International Classification of Diseases, Tenth Revision, 1992), reported a total of 9 deaths related to TAO, depicting male to female gender ratio of 2:1 and white to black ethnicity ratio of 8:1.

Race

Buerger disease is relatively less common in people of northern European descent. Natives of India, Korea, and Japan, and Israeli Jews of Ashkenazi descent, have the highest incidence of the disease.[5]

Sex

Though Buerger disease is more common in males (male-to-female ratio, 3:1), incidence is believed to be increasing among women, and this trend is postulated to be due to the increased prevalence of smoking among women.

Age

Most patients with Buerger disease are aged 20-45 years.

History Because a firm diagnosis of Buerger disease is difficult to establish, a number of different diagnostic

criteria have been proposed.[6] Olin asserts that the following criteria must be met for the diagnosis to be made with reasonable certainty:[7]

Age younger than 45 years Current (or recent) history of tobacco use Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers, or

gangrene) documented by noninvasive vascular testing Exclusion of 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

Most patients (70-80%) with Buerger disease present with distal ischemic rest pain and/or ischemic ulcerations on the toes, feet, or fingers, as depicted in the images below.[8, 9]

The feet of a patient with Buerger disease. Note the ischemic ulcers on the distal portion of the left great, second, and fifth toes. Though the patient's right foot is normal in gross appearance, angiography

demonstrated compromised arterial flow to both feet. Superficial thrombophlebitis of the great toe in a patient with Buerger disease.

Progression of the disease may lead to involvement of more proximal arteries, but involvement of large arteries is unusual.

Patients may also present with claudication of the feet, legs, hands, or arms and often describe the Raynaud phenomenon of sensitivity of the hands and fingers to cold.

Foot or arch claudication may be erroneously attributed to an orthopedic problem. Patients who seek medical attention late in the course of their disease may present with foot infections

and, occasionally, with florid sepsis.

Although classic Buerger disease affects the vessels of the extremities, a few cases of aortic, cerebral, coronary, iliac, mesenteric, pulmonary, and renal thromboangiitis obliterans have been reported.

Physical Patients with Buerger disease develop painful ulcerations and/or frank gangrene of the digits. The hands and feet of patients with the disease are usually cool and mildly edematous. Superficial thrombophlebitis (often migratory) occurs in almost half of patients with Buerger disease. Paresthesias (numbness, tingling, burning, hypoesthesia) of the feet and hands and impaired distal

pulses in the presence of normal proximal pulses are usually found in patients with the disease. More than 80% percent of patients present with involvement of 3-4 limbs. More recently a point-scoring system has been proposed by Papa to support or contest the diagnosis of

TAO using the following criteria. Distal extremity (feet, toes, hands, fingers) involvement Onset before age 45 Tobacco use Exclusion of atherosclerosis or proximal source of emboli Lack of hypercoagulable state Lack of definable arteritis (ie, progressive systemic sclerosis, giant cell arteritis) Classic arteriographic findings Involvement of digital arteries of finger or toes Segmental involvement (ie, "skip areas") Corkscrew collaterals No atherosclerotic changes Classic histopathologic findings Inflammatory cellular infiltrate within thrombus Intact internal elastic lamina Involvement of surrounding venous tissues

Table 1. Scoring system for the diagnosis of thromboangiitis obliterans (x) (Open Table in a new window)

Positive points

Age at onset Less than 30 (+2)/30-40 years (+1)

Foot intermittent claudication Present (+2)/ by history (+1)

Upper extremity Symptomatic (+2)/ asymptomatic (+1)

Migrating superficial vein thrombosis Present (+2)/ by history only (+1)

Raynaud Present (+2)/ by history only (+1)

Angiography; biopsy If typical both (+2)/ either(+1)

Negative points

Age at onset 45-50 (-1)/more than 50 years (-2)

Sex, smoking Female (-1)/ nonsmoker (-2)

Location Single limb (-1)/no LE involved (-2)

Absent pulses Brachial (-1)/femoral (-2)

Arteriosclerosis, diabetes, hypertension, hyperlipidemia Discovered after diagnosis 5.1-10 years (-1)/2.1- 5 years later (-2)

Table 2. Sum of points defines the probability of the diagnosis of thromboangiitis obliterans (Open Table in a new window)

Number of points Probability of diagnosis

0-1 Diagnostic excluded

2-3 Suspected, low probability

4-5 Probable, medium probability

6 or more Definite, high probability

CausesPropagating agents include cigarettes, as depicted in the image below, chewing tobacco, nicotine patches, and secondhand tobacco smoke (the latter two have been implicated as propagating agents of the disease only in former smokers).

Differential Diagnoses Antiphospholipid Antibody Syndrome and Pregnancy Atherosclerosis Diabetes Mellitus, Type 1 Diabetes Mellitus, Type 2 Frostbite Giant Cell Arteritis Gout Infrainguinal Occlusive Disease Peripheral Arterial Occlusive Disease Polyarteritis Nodosa Raynaud Phenomenon Reflex Sympathetic Dystrophy Scleroderma Systemic Lupus Erythematosus Takayasu Arteritis Thoracic Outlet Obstruction

Laboratory Studies No specific laboratory tests confirm or exclude the diagnosis of Buerger disease. The primary goal of a

laboratory workup in patients thought to have the disease is to exclude other disease processes in the differential diagnosis. Tests often used as markers for the diagnosis of systemic vasculitis, such as the acute-phase reactants, are negative in TAO. A complete serologic profile must be obtained.

CBC count with differential Liver function tests Renal function tests Urinalysis Glucose (fasting) Erythrocyte sedimentation rate C-reactive protein Antinuclear antibody Rheumatoid factor Complement Anticentromere antibody Scl-70 antibody Antiphospholipid antibodies

Imaging Studies

Angiography/arteriography Arteriographic abnormalities consistent with Buerger disease are sometimes seen in limbs that are not

yet clinically involved; therefore, arteriography of all 4 limbs may be required. The hallmark angiographic findings in patients with Buerger disease are nonatherosclerotic, segmental

occlusive lesions of the small- and medium-sized vessels (eg, digital, palmar, plantar, tibial, peroneal, radial, and ulnar arteries) with formation of distinctive small-vessel collaterals around areas of occlusion known as "corkscrew collaterals", as depicted in the image below. Such arteriographic findings suggest Buerger disease but are not pathognomonic because similar lesions can be observed in patients with scleroderma, CREST syndrome, systemic lupus erythematosus, rheumatoid vasculitis, mixed connective-tissue disease, antiphospholipid-antibody syndrome, and even diabetes mellitus.

This lower extremity arteriogram of the peroneal and tibial arteries of a patient with Buerger disease demonstrates the classic findings of multiple small- and medium-sized arterial occlusions with formation of compensatory "corkscrew collaterals."

Echocardiography: Always perform echocardiography in patients thought to have Buerger disease in order to exclude a proximal source of emboli as the cause of distal vessel occlusion.

Other Tests An abnormal Allen test result indicating distal arterial disease and establishing involvement of the upper

extremities in addition to the lower extremities helps differentiate thromboangiitis obliterans from atherosclerotic disease.

To perform the Allen test, the patient is instructed to make a fist, which exsanguinates the hand and fingers. The examiner's thumbs are then used to occlude the radial and ulnar arteries. The patient then opens the hand, after which the examiner releases the pressure on the ulnar artery while the radial artery remains compressed.

Prompt return of color to the hand indicates patency of the ulnar artery (ie, a normal or negative test result). The patency of the radial artery can then be tested by repeating the maneuver but with the pressure on the radial artery released while the ulnar artery remains compressed.

Failure of the hand to promptly refill with blood indicates occlusion of the respective artery distal to the wrist (ie, an abnormal or positive test result). While an abnormal result can be present in other types of small-vessel occlusive disease of the hands, a positive Allen test finding in a young smoker with leg ulcerations is highly suggestive of Buerger disease.

Histologic FindingsOlin contends that a biopsy is rarely needed unless the patient presents with unusual characteristics, such as large-artery involvement, or age older than 45 years.

In its acute phase, Buerger disease is characterized by highly cellular, segmental, occlusive, inflammatory thrombi, with minimal inflammation in the walls of affected blood vessels. Secondary spread from the affected small- and medium-sized arteries to contiguous veins and nerves is often observed. Microscopically, the polymorphonuclear leukocyte–predominant inflammatory cellular aggregate may form microabscesses and multinucleated giant cells.

In the subacute phase, intraluminal thrombosis progressively organizes, but it may defer to vascular recanalization.[10]

The end-stage phase of the disease is characterized by mature thrombus and vascular fibrosis.

In all 3 stages, the integrity of the normal structure of the vessel wall, including the internal elastic lamina, is maintained. This distinguishes thromboangiitis obliterans from arteriosclerosis and from other types of systemic vasculitis, in which disruption of the internal elastic lamina and the media can be extensive.

Medical CareAbsolute discontinuation of tobacco use is the only strategy proven to prevent the progression of Buerger disease. Smoking as few as 1 or 2 cigarettes daily, using chewing tobacco, or even using nicotine replacements may keep the disease active.[11]

Except for absolute tobacco avoidance, no forms of therapy are definitive. Treatment with intravenous iloprost (a prostaglandin analogue), an expensive therapy unavailable in the United States, has been shown to be somewhat effective in improving symptoms, accelerating resolution of distal extremity trophic changes, and reducing the amputation rate among patients with Buerger disease. Intravenous iloprost use is probably of greatest value in slowing progressive tissue loss and reducing the need for amputation in patients with critical limb ischemia during the period when they first discontinue cigarette smoking.

The use of thrombolytic therapy in the treatment of Buerger disease has been proposed, but the data for this treatment remain inconclusive and the treatment is thus considered experimental. Recently, Isner and colleagues reported improved healing of ischemic ulcers and relief of rest pain in a small series of patients with Buerger disease using intramuscular gene transfer of vascular endothelial growth factor.[12]

The following strategies are important in prevention of complications from Buerger disease:

Use of well-fitting protective footwear to prevent foot trauma and thermal or chemical injury Early and aggressive treatment of extremity injuries to protect against infections Avoidance of cold environments Avoidance of drugs that lead to vasoconstriction

Surgical CareGiven the diffuse segmental nature of thromboangiitis obliterans and the fact that the disease primarily affects small- and medium-sized arteries, surgical revascularization for Buerger disease is usually not feasible and is extremely rare in the United States. However, make every effort to improve distal arterial flow in patients with Buerger disease, and consider autologous vein bypass of coexistent large-vessel atherosclerotic stenoses in patients with severe ischemia who have an acceptable distal target vessel.

Other proposed surgical treatments for Buerger disease are as follows:o Omental transfero Sympathectomyo Spinal cord stimulator implantation

Ultimate surgical therapy for refractory Buerger disease (in patients who continue smoking) is distal limb amputation for nonhealing ulcers, gangrene, or intractable pain. Avoid amputation when possible, but, if it is necessary, perform the operation in a way that preserves as much of the limb as possible.

Consultations Rheumatologists

Vascular surgeons Smoking cessation counselors

DietNo dietary restrictions are needed. Diet has not been shown to affect the course of the disease.

ActivityEncourage cardiovascular exercise. Activity should be restricted by symptoms only.

Medication SummaryOther than the experimental use of iloprost and thrombolytics (as discussed previously), the use of antibiotics to treat infected ulcers, and palliative treatment of ischemic pain with nonsteroidal and narcotic analgesics, all other forms of pharmacologic treatment have been generally ineffective in the treatment of Buerger disease, including steroids, calcium channel blockers, reserpine, pentoxifylline, vasodilators, antiplatelet drugs, and anticoagulants.

Proceed to Follow-up 

Further Inpatient Care Indications for admission of patients with Buerger disease include the following:

Surgery Parenteral pharmacological treatment of infection or pain that is refractory to oral medical therapy Intensive behavioral modification therapy for patients unable to achieve smoking cessation at home

Further Outpatient CareOutpatient management is generally appropriate for patients with Buerger disease and should include frequent follow-up examination by a physician or wound-care specialist.

Inpatient & Outpatient MedicationsOral nonsteroidal and narcotic analgesics can be administered to palliate ischemic pain, and appropriate oral antibiotics can be used to treat mild distal extremity ulcers.

TransferOther than in the rare Buerger patient with ascending limb infection and associated sepsis, transfer is generally safe.

Deterrence/PreventionPatients must be counseled to never smoke.

Complications Ulcerations Gangrene Infection Need for amputation Rare occlusion of coronary, renal, splenic, or mesenteric arteries

PrognosisA striking dichotomy is observed with regard to the prognosis of patients with Buerger disease, which is dependent upon whether absolute avoidance of tobacco is achieved. Among patients with who quit smoking, 94% avoid amputation; among patients who quit smoking before gangrene develops, the amputation rate is near 0%. This is in stark contrast to patients who continue smoking, for whom there is a 43% chance that an amputation will be required sometime during a 7- to 8-year period. It is not uncommon for patients with Buerger disease who continue to smoke to require multiple amputations, and reports have even been made of patients who have required bilateral above-knee and above-elbow

amputations. While smoking cessation generally removes the need for limb amputation, patients may continue to have claudication or Raynaud phenomenon even after complete discontinuation of tobacco use.

Patient Education Patients with Buerger disease must be repeatedly implored to quit smoking and can be reassured that if

they are able to discontinue tobacco use, the disease will remit and amputation will be avoided. Physicians should counsel patients that the level of tobacco avoidance required to achieve resolution of

their disease often necessitates that they even rigorously limit their exposure to secondhand smoke. This can be extremely difficult for patients who live with another smoker, and it is therefore not unreasonable to consider referring such patients (and their loved ones) to multidisciplinary smoking cessation programs.

Patients with Buerger disease who are bedridden should be educated about the importance of protective heel pads or foam boots.

For excellent patient education resources, visit eMedicineHealth's Healthy Living Center and Lung and Airway Center. Also, see eMedicineHealth's patient education article Cigarette Smoking.

http://emedicine.medscape.com/article/460027-followup#showall

First DescriptionThis 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 Buerger’s Disease

is thromboangiitis obliterans.

Who gets Buerger’s Disease (the “typical” patient)?The classic Buerger’s Disease patient is a young male (e.g., 20–40 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. Buerger’s 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 Buerger’s DiseaseThe initial symptoms of Buerger’s 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 Raynaud’s 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 Buerger’s

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

Buerger’s, 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 Buerger’s Disease?The association of Buerger’s Disease with tobacco use, particularly cigarette smoking,

cannot be overemphasized. Most patients with Buerger’s 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 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.

Pictured below, are a patient’s fingertips that have developed gangrene. This is a very

painful condition which sometimes requires amputation of the affected area.

How is Buerger’s diagnosed?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 only one treatment known to be effective: complete smoking

cessation — see below).

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 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 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.

Treatment and Course of Buerger’sIt is essential that patients with Buerger’s disease stop smoking immediately and

completely. This is the only treatment known to be effective in Buerger’s 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.

http://www.hopkinsvasculitis.org/types-vasculitis/buergers-disease/

Smoking and Buerger's DiseaseWhat Is Buerger's Disease?

Buerger’s disease affects blood vessels in the arms and legs. Blood vessels swell, which can prevent

blood flow, causing clots to form. This can lead to pain, tissue damage, and even gangrene (the death

or decay of body tissues).1 In some cases, amputation may be required.2

The most common symptoms of Buerger’s disease are:2

Pale, red, or bluish hands or feet

Cold hands or feet

Pain in the hands and feet; may be severe

Pain in the legs, ankles, or feet when walking—often located in the arch of the foot

Skin changes, painful sores, or ulcers on the hands or feet

Top of PageHow Is Smoking Related to Buerger's Disease?

Almost everyone with Buerger’s disease smokes cigarettes. However, Buerger’s disease can occur in

people who use other forms of tobacco, like chewing tobacco. People who smoke 1½ packs a day or

more are most likely to develop Buerger's disease.3

Researchers are working to understand how tobacco increases the risk for Buerger's disease. One idea

is that chemicals in tobacco irritate the lining of the blood vessels and cause them to swell.3

Top of PageHow Can Buerger's Disease Be Prevented?

If you want to prevent getting Buerger’s disease, don’t smoke or use any other tobacco products.1

Top of PageHow Is Buerger's Disease Treated?

There is no cure for Buerger’s disease. The only way to keep Buerger’s disease from getting worse is to

stop using all tobacco products. Medicines don’t usually work well to treat the disease. The best they

can do is to control the symptoms.2

Surgery may help restore blood flow to some areas.1 It may be necessary to amputate the hand o

http://www.cdc.gov/tobacco/campaign/tips/diseases/buergers-disease.html

Buerger's DiseaseImportant It is possible that the main title of the report Buerger's Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) anddisorder subdivision(s) covered by this report.

Synonyms

Inflammatory Occlusive Peripheral Vascular Disease Occlusive Peripheral Vascular Disease Thromboangiitis Obliterans TAO

Disorder Subdivisions

None

General Discussion

Buerger's disease, also known as thromboangiitis obliterans, is a rare disorder that, in most cases, affects young or middle-aged male cigarette smokers. It 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. In most cases, the first symptom is extreme pain of the lower arms and legs while at rest. Affected individuals may also experience cramping in the legs when they walk that, in rare cases, may cause limping (claudication). In addition, affected individuals may have sores (ulcers) on the extremities, numbness and tingling and a lack of normal blood flow to the fingers and/or toes when exposed to cold temperatures (Raynaud's phenomenon), and/or inflammation and clotting of certain veins (thrombophlebitis). In severe cases, individuals with Buerger's disease may exhibit tissue death (gangrene) of affected limbs. The exact cause of Buerger's disease is not known; however, most affected individuals are heavy tobacco users.

Resources

http://www.webmd.com/a-to-z-guides/buergers-disease

Pathophysiology of Buerger's Disease

Pathophysiology

Smoking is very closely related to Buerger's disease and smoking history is one of the critera 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 identified by measuring levels of nicotine, 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 (www.indiandoctors.com).

Most patients with Buerger’s 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 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 (www.karr.net).

Mogotlane (2005) said that nicotine in cigarette acts as a vasoconstrictor so there will be less oxygen supply to the extremities, and in addition, is thought to alter the surface properties of platelets so that they become more stick, increasing the risk of clots formation causing further damage.

Although this condition is different from atherosclerosis, Buerger’s disease in older patients may also be followed by atherosclerosis of the larger vessels after involvement of the smaller vessels. The patient’s ability to walk may be severely limited. Patients are at higher risk for non healing wounds because of impaired circulation (Smeltzer and Bare, 2004).

Onset of the disease is gradual and first occurs in the feet or hands. Inflammation occurs in small and medium-sized arteries and veins near the surface of the limb. In advanced cases, blood vessels in other parts of the body may be affected. There is a progressive decrease in the blood flow to the affected areas. The pulse in arteries of the feet is weak or undetectable. The lack of blood flow can lead to gangrene, which is decay of tissue due to restricted blood supply (Zhang, 2008).

Symptoms. The thromboangiitis obliterans (Buerger’s disease presents with a triad of symptoms and signs: digital arteries occlusion, Raynaud’s phenomenon, and migrating superficial vein thrombophlebitis (Lilly, 2007).

Early symptoms include decrease in the blood supply (arterial ischemia) and superficial (near the skin surface) phlebitis. The main symptom is pain in the affected areas is the outstanding manifestation. A cold sensitivity in the hands with color changes, similar to that seen in Raynaud's disease, can develop and may be another early manifestation. In this case, the hands turn color--white, blue, and then intense rubor or reddish blue discoloration (Zhang, 2008; Smeltzer and Bare, 2004).

Claudication type of pain is common with pain in the arch of the foot. Various types of paresthesias may occur. Pulsations in the posterior tibial and dorsalis pedis arteries are weak or absent. In advance cases, the extremities may be abnormally red or cyanotic, particularly when dependent (Black and Hawks, 2005).

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