Buerger Disease

Buerger Disease

Buerger Disease | Johns Hopkins Medicine

Linkedin Pinterest Blood and Bone Marrow Buerger disease is one of many types of vasculitis. This is inflammation of small and medium size blood vessels. Buerger disease causes a tightening, or a blockage, of the blood vessels in your feet and hands. When blood doesn’t flow well to your hands and feet, especially during activity, you may have pain and tissue damage.

In the worst cases, sores (ulcers) appear on your fingers and toes due to poor circulation to the skin and tissue. The ulcers can become infected and cause gangrene. In a small number of people, Buerger disease reduces blood flow to the heart, belly, or brain. People at the greatest risk for Buerger disease are those who are heavy smokers.

Men of Asian or Eastern European descent, who are between ages 20 and 40 seem to be especially at risk. It has also been identified cigar smokers, marijuana users, and those who use smokeless tobacco such as chewing tobacco and snuff. It is a rare disorder, especially in countries where the use of tobacco has declined. Healthcare providers are not sure what causes Buerger disease.

It is a rare type of vasculitis.

Symptoms of Buerger disease include:

  • Pain, tenderness or burning feeling in your lower legs or feet when walking
  • Pain or tenderness in your hands or forearms
  • Blood clots
  • Ulcers on your toes and fingers
  • Change in the color of the skin on the fingers and toes to pale, redness, and sometimes bluish in color

How is Buerger disease diagnosed?

To make a diagnosis, your healthcare provider will consider:

  • Your overall health and medical history
  • Your symptoms
  • Lifestyle choices, such as whether you smoke
  • A physical exam
  • Blood tests
  • Imaging tests to assess tissue damage

How is Buerger disease treated?

Your healthcare provider will figure out the best treatment :

  • How old you are
  • Your overall health and medical history
  • How sick you are
  • How well you can handle specific medicines, procedures, or therapies
  • How long the condition is expected to last
  • Your opinion or preference

There is no cure for Buerger disease. Treatment may depend on how far your disease has progressed. It will focus on trying to improve blood flow to the affected area or to reduce pain. Choices include:

  • Stopping all smoking and other forms of tobacco use, including electronic cigarettes.
  • Having surgery to bring blood to the affected tissues
  • Other types of surgery to cut the nerves to the tissue to reduce pain or treat damaged extremities

Some medicines help to relax the blood vessels and improve blood flow to the tissue.

What are the complications of Buerger disease?

Possible complications from Buerger disease include:

  • Changes in the way you walk or move due to pain
  • Ulcers
  • Tissue damage or tissue death (gangrene)
  • Amputation

What can I do to prevent Buerger disease?

The goal is to prevent the disorder from getting worse by

  • Stopping smoking and avoiding all tobacco products.
  • Avoid long periods of time spent in cold temperatures.
  • Dressing warmly in cold temperatures.

How can I manage Buerger disease?

Follow your healthcare provider’s advice for taking care of yourself. The best way to stop or slow Buerger disease is to stop using tobacco completely. Talk with your healthcare provider if you need help quitting tobacco.

When should I call my healthcare provider?

Talk to your healthcare provider if you have:

  • Pain in your lower legs, feet, hands, or forearms that continues to get worse
  • Sores on your hands or feet that do not heal
  • Signs of infection in the affected areas

Key points about Buerger disease

  • Buerger disease causes the blood vessels to narrow and severely limits the blood flow to the area, usually in the hands and feet.
  • It is a rare disorder that can’t be prevented. It affects men more frequently than women.
  • Quitting smoking and stopping use of any forms of tobacco or nicotine is important to reduce the risk of complications.  

Next steps

Tips to help you get the most from a visit to your healthcare provider:

  • Know the reason for your visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • Bring someone with you to help you ask questions and remember what your provider tells you.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
  • Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
  • Ask if your condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if you do not take the medicine or have the test or procedure.
  • If you have a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your provider if you have questions.

Source: https://www.hopkinsmedicine.org/health/conditions-and-diseases/buergers-disease

Buerger’s Disease (Thromboangiitis Obliterans)

Buerger Disease | Johns Hopkins Medicine

Buerger’s disease is a rare illness that inflames and blocks blood vessels in your arms and legs. This stops blood flow and leads to clots that affect your hands and feet.

Doctors also call it thromboangiitis obliterans, or TAO.

There’s no cure for Buerger’s disease. Almost everyone who gets it uses tobacco, including cigarettes, cigars, chewing tobacco, and snuff. Quitting can make the symptoms improve or go away completely. If you don’t quit, you might have severe tissue damage. You could even lose your fingers, toes, or parts of your limbs.

Doctors aren’t sure exactly what causes this condition. Your genes might play a role. Some people may just be more ly to get it.

Some experts think that certain chemicals in tobacco might irritate your blood vessels, and that’s what makes them swell. Others think tobacco triggers your immune system to attack your blood vessels they’re bad germs.

Usually, the first sign of Buerger’s disease is pain in your hands or feet that spreads to your arms and legs. It may be severe. You can get it when you’re active (called claudication) or resting. It can be worse when you’re cold or stressed.

You might also notice changes in your hands and feet. They may:

  • Feel cold, numb, or tingly
  • Look pale, red, or blue in color
  • Have thin, shiny skin
  • Have less hair than usual

Your fingers and toes might:

  • Get painful open sores
  • Turn pale when you’re cold (Raynaud’s phenomenon)

You could also have swelling along a vein just under your skin. This is usually a sign of a blood clot. See your doctor right away.

Over time, Buerger’s disease can slow — and completely stop — the blood flow to your fingers and toes. This can cause gangrene, which means the skin and tissue in your toes and fingers start to die. They go numb and turn blue or black.

If you have gangrene, your doctor usually has to cut off the affected area.

In rare cases, Buerger’s disease can cause a stroke or heart attack.

No single test can check for Buerger’s disease. Your doctor will start by asking about your tobacco use and your symptoms.

They may do tests to check your blood flow and rule out other diseases that cause similar symptoms. For example, peripheral artery disease also causes pain in your legs, but it’s caused by plaque building up in your arteries, not inflammation.

You may also have:

  • The Allen test. This is a basic blood flow test. First, you squeeze your hand into a hard fist, which pushes blood your hand. Your doctor then presses on the arteries of your wrist to slow the flow of blood back into your hand. At this point, your hand will lose color. When you open your hand, your doctor releases the pressure over the artery on one side of your wrist and then the other side. If it takes a while for your hand to go back to its usual color, it may be a sign of Buerger’s disease.
  • Angiogram. This is a type of X-ray that checks for blocked blood vessels in your arms and legs. Your doctor places a thin tube, called a catheter, into your artery. They pump dye into the artery and quickly take X-rays to get a look at your blood vessels. A CT or MRI scan can make similar pictures.
  • Blood tests. These help your doctor rule out other diseases. They may want to check for conditions diabetes, lupus, and those that cause blood clots.

Quitting tobacco is the only way to limit the effects of Buerger’s disease. Even a few cigarettes a day can make it worse.

Other treatments may help boost blood flow and ease pain, including:

  • Medications such as vasodilators to widen your blood vessels or others to dissolve clots
  • Walking exercises
  • Intermittent compression of your arms and legs
  • Surgery to cut nerves to an affected area (sympathectomy)

Your doctor might need to cut off part or all of an area with infection or gangrene (amputation).

Researchers are also studying other treatments. Proteins called growth factors might speed healing and ease pain. Injections of stem cells, which can grow into other kinds of cells, may help your body make new blood vessels.

Your outlook depends on whether you quit tobacco. Of patients who keep using it, about 43% will have an amputation within 8 years of diagnosis. Of those who quit, about 6% have an amputation. That percentage is close to 0 in people who are diagnosed early on.

If you’re having a hard time quitting, talk to your doctor about programs that might help.

SOURCES:

CDC: “Health Effects of Cigarette Smoking.”

CDC: “Smoking and Buerger’s Disease.”

Mayo Clinic: “Buerger’s Disease.”

The Johns Hopkins Vasculitis Center: “Buerger’s Disease.”

UC Davis Vascular Center: “Buerger’s Disease (Thromboangitis Obliterans).”

National Organization of Rare Disorders: “Buerger’s Disease.”

Vasculitis Foundation: “Buerger’s Disease.”

Penn Medicine: “Buerger’s Disease (Thromboangitis Obliterans).”

UpToDate: “Thromboangiitis Obliterans (Buerger’s disease).”

NYU Langone Health: “Types of Vasculitis.”

ePlasty, Open Access Journal of Plastic Surgery: “Thromboangiitis Obliterans (Buerger’s Disease).”

National Center for Advancing Translational Sciences Genetic and Rare Diseases Information Center: “Buerger disease.”

Medscape: “Thromboangiitis Obliterans (Buerger Disease).”

© 2020 WebMD, LLC. All rights reserved.

Source: https://www.webmd.com/a-to-z-guides/what-is-buergers-disease

Johns Hopkins Vasculitis Center

Buerger Disease | Johns Hopkins Medicine

There are approximately 20 different disorders that are classified as “vasculitis”. “Angiitis” and “Arteritis” are both synonyms for vasculitis, literally meaning “inflammation within blood vessels” or “inflammation in arteries.”  Because there are so many types of vasculitis, the group is sometimes referred to in the plural: vasculitides (pronounced “vas que lit’ i deez”).

There are many different types of diseases that belong to this category. Although the diseases are similar in some ways, they often differ with respect to which organs are affected, which medications are used to treat them, and other characteristics.

Behcet’s Disease 
Characterized by the triad of mouth ulcers, genital ulcers, and eye inflammation. However, other organ systems may also be affected.

Buerger’s Disease (Thromboangiitis Obliterans)
Mainly affects smokers, leading to decreased blood flow to the hands and feet.

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg Strauss)
Associated with asthma, nasal polyps, sinusitis, elevated eosinophil counts, and vasculitis. EGPA has a tendency to involve lungs, peripheral nerves, skin, kidneys, and heart.

Cryoglobulinemia
Characterized by recurrent purpura (red dots) on the lower extremities and may be associated with hepatitis C virus infections or paraproteinemias.

Giant Cell Arteritis
This disease was previously named “temporal arteritis”, and is the most common type of vasculitis in adults in North America. It is a large vessel vasculitis that affects people over the age of 50 (although most individuals affected are 70-80 years of age). It can be characterized by fever, headache, and jaw/scalp pain.

Henoch-Schönlein Purpura
This disorder is often followed after an upper respiratory tract infections and is often, but not necessarily, self-limited.

Microscopic Polyangiitis
A systemic vasculitis affecting small and medium–sized blood vessels associated with the autoantibody, ANCA.

Polyarteritis Nodosa
The prototype of systemic vasculitis, involving many different organ systems and focussed on medium–sized arteries.

Polymyalgia Rheumatica
A syndrome of pain and stiffness localized to the shoulders and hips which often occurs in association with Giant Cell Arteritis.

Rheumatoid Vasculitis
A vasculitis that sometimes complicates the course of rheumatoid arthritis (RA). Usually occurs in patients with a history of severe RA.

Takayasu’s Arteritis
A large vessel vasculitis that affects the aorta, its major branches to the extremities, and sometimes internal organs. Usually occurs in young women (< age 50 years).

Granulomatosis with Polyangiitis (GPA, formerly known as Wegener’s)
A systemic disease that involves the lungs, kidneys, upper respiratory tract, and other organs and associated with the autoantibody, ANCA.

Source: https://www.hopkinsvasculitis.org/types-vasculitis/

Thromboangiitis Obliterans (Buerger Disease) Clinical Presentation: History, Physical Examination, Complications

Buerger Disease | Johns Hopkins Medicine

Patients with TAO can develop painful ulcerations (see the image below) or frank gangrene of the digits. The hands and feet of patients with the disease are usually cool and mildly edematous.

Tobacco smoke stains on male patient's fingers suggest diagnosis of thromboangiitis obliterans (Buerger disease). Patient presented with small, painful ulcers on tips of thumb and ring finger.

Superficial thrombophlebitis (often migratory) occurs in almost half of patients with TAO (see the image below). 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 severe disease. More than 80% of patients present with involvement of three or four limbs.

Superficial thrombophlebitis of great toe in patient with thromboangiitis obliterans (Buerger disease).

In 1996, Papa et al proposed a point-scoring system to support or contest the diagnosis of TAO on the basis of the following criteria [11] :

  • Distal extremity (feet, toes, hands, or fingers) involvement
  • Onset before age 45 years
  • Tobacco use
  • Exclusion of atherosclerosis or proximal source of emboli
  • Absence of a hypercoagulable state
  • Absence of definable arteritis (eg, progressive systemic sclerosis or 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

The scoring system is applied as indicated in Table 1 and Table 2 below.

Table 1. Scoring System for Diagnosis of Thromboangiitis Obliterans [11] (Open Table in a new window)

Positive Criterion Positive Points
Age at onset < 30 y (+2)30-40 y (+1)
Foot intermittent claudication Present (+2)By history only (+1)
Upper extremity Symptomatic (+2)Asymptomatic (+1)
Migrating superficial thrombophlebitis Present (+2)By history only (+1)
Raynaud phenomenon Present (+2)By history only (+1)
Angiography; biopsy If typical, both (+2)Either(+1)
Negative Criterion Negative Points
Age at onset 45-50 y (−1)>50 y (−2)
Sex; smoking Female (−1)Nonsmoker (−2)
Location Single limb (−1)No lower extremity involved (−2)
Absent pulses Brachial (−1)Femoral (−2)
Arteriosclerosis, diabetes, hypertension, hyperlipidemia Discovered 5.1-10 y after diagnosis (−1)Discovered 2.1-5 y later (−2)

Table 2. Numerical Scores Defining Probability of Diagnosis of Thromboangiitis Obliterans (Open Table in a new window)

Score Probability of Diagnosis
0-1 Diagnosis excluded
2-3 Diagnosis suspected (low probability)
4-5 Diagnosis probable (medium probability)
≥6 Diagnosis definite (high probability)

Source: https://emedicine.medscape.com/article/460027-clinical

An Overview of Buerger’s Disease

Buerger Disease | Johns Hopkins Medicine

Buerger’s disease (also known as thromboangiitis obliterans) is a rare condition and type of vasculitis where inflammation, swelling, and blood clotting occurs in the small and medium-sized arteries and veins surrounding the hands and feet. Over time, this narrowing of the arteries and veins leads to damaged skin tissue, causing infection and in severe cases gangrene or complete tissue death.

Illustration by Brianna Gilmartin, Verywell

In cases of extreme gangrene, this may lead to amputation of the body part affected.

Since most cases of Buerger’s disease occur in smokers, it is most prevalent in countries whose residents use tobacco heavily such as in the Mediterranean, Middle East, and Asia.

The disease is named after Leo Buerger, an Austrian American pathologist and surgeon who most accurately identified the condition in 1908 at Mount Sinai in New York City.

Common symptoms of Buerger’s disease include:

  • pale, red, or blue-tinted hands and feet.
  • cold hands or feet.
  • severe pain in hands and feet.
  • pain in the lower arms and legs while at rest due to limited blood supply.
  • pain when walking in the legs, ankles, or feet (most commonly in the arch of the foot).
  • sores or ulcers on the hands and feet that are often painful.
  • lack of blood flow to fingers and toes in cold weather (known as Raynaud’s phenomenon).
  • if arteries and veins in the intestine are affected, extreme heaviness and pain in the abdomen.
  • weight loss, for issues surround arteries and veins in the intestine.

Episodes of Buerger’s disease happens in short periods, with symptoms lasting usually one to four weeks, then temporarily subsiding. The disease presents primarily in males between the ages of 20 to 40 years old. However, incidences of women being diagnosed with the disease are starting to spike, most ly due to the increase of cigarette use among women.

Due to the lack of blood flow Buerger’s disease causes, symptoms are mainly confined to extremities the fingers and toes, leaving internal organs untouched.

Almost all patients diagnosed with Buerger’s disease smoke cigarettes or use other forms of tobacco. While researchers are unclear on the exact link between tobacco and Buerger’s disease, it’s thought that the chemicals in tobacco irritate the lining of the blood vessels, causing inflammation and swelling.

For this reason, those who smoke hand-rolled tobacco are at the greatest risk due to the potency and lack of filtering of the tobacco.

Smokers who smoke a pack and a half a day are at the greatest risk of being diagnosed with the disease.

Other less prevalent causes of Buerger’s disease include genetic predispositions and in rare cases, an autoimmune disease where the body attacks healthy tissue for unknown reasons. Long-term, chronic gum disease can also lead to Buerger’s disease.

In order to be properly diagnosed with Buerger’s disease, a number of other diseases that cause limited blood flow are often ruled out first.

Your health care provider will do a thorough examination to determine that these other disorders are not the cause of inflammation and swelling in the arteries and veins, as the treatment plans for disorders that are not Buerger’s disease are very different in nature.

In addition to an exam, patients must provide their health care providers with an up-to-date history of their tobacco use. With this information, health care providers will perform a blood test and possibly an angiography—a type of cardiac catheterization where dye is injected to see the construction of blood vessels via X-ray—though there are also noninvasive options.

Your health care provider will also ly conduct an Allen test to check your blood flow in the arteries linked to your hand. With this test, you’ll make a tight fist to force the blood your hand.

Your doctor will then press on the arteries on each side of your wrist to slowly flow blood back into your hand, noting the color of your hand during this process.

You’ll then open your hand as your doctor releases the pressure on each artery (doing one at a time).

The Allen test will show how quickly the circulation in your hand will return your skin to its normal color. This will give insight to the health of your arteries, as a slow blood flow may indicate Buerger’s disease.

What a patient thinks is Buerger’s disease is often another form of vasculitis or related disease, such as scleroderma or Takayasu's Arteritis. Scleroderma is a rheumatic disease characterized by inflammation and pain in the muscles, joints, and connective tissue.

Takayasu’s Arteritis is a separate rare disorder that’s classified by inflammation in large arteries, such as the heart and lungs, which then restricts blood flow throughout the body.

Both of these conditions have similar symptoms to Buerger’s disease.

This makes it important for your health care provider to have your entire health history when trying to pinpoint the correct disease causing blood flow issues.

The symptoms of Buerger’s disease will only cease when a patient stops smoking or using other forms of tobacco products.

In some cases, quitting tobacco may result in a complete remission of the disease and its side effects.

Medication will not treat Buerger’s disease but can often help control the symptoms of the disease (though anti-inflammatories and blood thinners have varied degrees of success depending on the patient).

More extensive measures such as surgery may be needed to help improve blood flow to certain areas. This includes possible amputation if certain areas of the body have extreme and irreversible tissue death.

However, most patients who stick to smoking cessation will not have to resort to more invasive procedures surgery. For rare cases where patients with Buerger’s disease are not smokers, medication may be used to help dilate blood vessels, reduce blood clots, improve blood flow, or even stimulate the growth of new blood vessels (a more recent experimental approach to the disease).

Regular and frequent compression to the arms, legs, and other affected extremities to improve blood flow may also be part of the treatment plan.

Getting diagnosed with Buerger’s disease can be scary, but patients should take comfort in the fact that quitting smoking and other tobacco products has enormous success rates of halting the disease.

Among the other health reasons to stop smoking immediately, avoiding the symptoms and side effects of Buerger’s disease should be top of mind for those thinking about smoking cessation and living a healthier lifestyle. 

Source: https://www.verywellhealth.com/buergers-disease-overview-4571052

Rare disease: Buerger’s disease (Thromboangiitis obliterans): a diagnostic challenge

Buerger Disease | Johns Hopkins Medicine

Buerger´s disease or Thromboangiitis obliterans is a segmental inflammatory disease that affects the vessels and nerves of the extremities. It usually affects men below 45 years old and correlates with tobacco, as a predisposing factor.

The authors present the case of a 34-year-old male, with ulcers in the fingertips with progressive worsening: acrocyanosis, slow healing, necrosis and finally loss of substance. Dorsalis pedis and posterior tibial pulses were not palpable. Personal history of heavy smoking was (20 pack-years).

The angiography revealed proximal occlusion of the left posterior tibial and interosseal arteries, with distal circulation by the anterior tibial artery.

He was submitted to disarticulation of the second left toe and therapy with pentoxifyline and iloprost infusion, calcium antagonist, antiplatelet drugs, statin and low molecular weight heparin (later replaced by oral anticoagulation). Improvement was seen of active vascular lesions and pain symptoms.

Buerger’s disease or Thromboangiitis obliterans is a type of vasculitis in young, mostly male subjects- remains strangely linked to smoking, which determines its ocurrence, progression and prognosis by yet unknown mechanisms.1 Sometimes it presents with multisystemic complaints.

The diagnosis and treatment of this entity is challenging, since it requires the exclusion of many other causes and a multidisciplinary approach. An illustrative case-report and literature review is presented.

A 34-year-old black male presented in the medicine clinics with a 6 year history of ulcers in the fingertips of both hands and feet with progressive worsening: acrocyanosis, slow healing, necrosis and finally loss of substance.

Raynaud’s phenomenon was absent. Sympaticectomy was unsuccessfully tried. He also referred recurrent episodes of ‘migratory thrombophlebitis’, preferentially involving the legs bilaterally and left forearm.

A biopsy made in the past was consistent with local thrombophlebitis.

The patient had a known history of smoking (20 pack-year), controlled asthma, sinusitis and primary syphilis (1995). No other vascular risk factor was detected, namely diabetes, dyslipidemia or drug abuse.

On clinical examination, the patient presented a cyanotic ulcerated lesion involving the two distal thirds of the second left toe (figure 1).

The patient had an ulcerated lesion on the first right toe with purulent discharge.

Healed lesions with loss of substance in the first left toe and second and fourth fingers of right hand were present (figures 2 and ​3). Dorsalis pedis and posterior tibial pulses were absent.

The differential diagnoses were: systemic embolisation or thrombophilia, Buerger’s disease, vasculitis of large and medium-sized vessels, atherosclerotic arteriopathy (less ly in this age group) and systemic lupus erythematosus- related vasculitis.

The study for thrombophilia and immunology was negative (table 1). A possible source of systemic embolism was excluded by transthoracic echocardiogram and carotid-vertebral Doppler. The viral (HIV, hepatitis B virus and hepatitis C virus) and syphilitic serologies were negative. The thoracoabdominopelvic CT was unremarkable.

The nailfold capillaroscopy was suggestive of systemic vascular disease, with active capillaritis. The angiography (abdominal, upper and lower limbs) revealed proximal occlusion of the left posterior tibial and interosseal arteries, with distal circulation by the anterior tibial artery (figure 4).

A vascular surgery consult excluded the need for a revascularisation procedure (endovascular /surgery).

Relevant laboratory parameters.

ParameterValue
Haemoglobin 15, 9 (13, 5–18 g/dl
Leucocytes/neutrophils 13300 (4–10) mm3; 60, 7% (37–72)
C reactive protein 2, 7 (

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3176380/