Thromboangiitis obliterans

Buerger disease, Buerger’s disease, Winiwarter-Buerger disease, presenile gangrene

Thromboangiitis obliterans is a rare disease that causes blood vessels in the arms and legs to become inflamed and blocked, leading to severe pain and tissue damage. Almost everyone who develops this condition uses tobacco, and quitting all tobacco use is the only proven way to stop the disease from getting worse.

Table of contents

• What is thromboangiitis obliterans?
• Which parts of the body are affected?
• Who gets this disease?
• What causes thromboangiitis obliterans?
• Symptoms and warning signs
• Possible complications
• How is it diagnosed?
• Treatment options
• What to expect

What is thromboangiitis obliterans?

Thromboangiitis obliterans, also known as Buerger disease, is a rare condition that affects the blood vessels in your arms and legs. The disease causes inflammation, which means the blood vessels become swollen and irritated. This inflammation makes it hard for blood to flow through them^[1][2].

When blood vessels become inflamed, blood clots can form inside them. These clots create obstacles that block blood flow even more. As a result, the tissues in your fingers and toes do not get enough blood and oxygen. Over time, this lack of blood can damage or destroy skin tissue, which may lead to infection and gangrene (death of body tissue)^[1][3].

Unlike other blood vessel diseases, thromboangiitis obliterans is not caused by a buildup of fatty deposits in the arteries. Instead, it involves a progressive inflammation that affects small and medium-sized arteries and veins. The disease was first described in 1879 and was later named after Leo Buerger, who published detailed findings about the condition in 1908^[3].

Which parts of the body are affected?

• Hands
• Feet
• Fingers
• Toes
• Arms
• Legs

The disease usually first appears in the feet and may eventually affect the blood vessels of the hands. The lower limbs are more commonly affected than the upper limbs. The condition targets small and medium-sized blood vessels, with arteries being more affected than veins^[1][5].

Who gets this disease?

Thromboangiitis obliterans is a rare condition. In the United States, out of every 100,000 people, only 12 to 20 have this disease. The number of cases has been decreasing as fewer people smoke^[2][3].

The disease typically affects people between 20 and 50 years old, with most patients being aged 20 to 45 years. It does not occur in children or elderly people. The condition is more common in men, with a male-to-female ratio of about 3 to 1. However, the number of cases among women has been increasing, likely because more women are smoking^[3][7].

The disease is less common in people of northern European descent. The highest rates are found in Israeli Jews of Ashkenazi descent and natives of India, Korea, and Japan. It also affects more people in the Middle East, Asia, the Mediterranean, and Eastern Europe^[3][5].

What causes thromboangiitis obliterans?

The exact cause of thromboangiitis obliterans is not known, but tobacco use is required for both starting and continuing the disease. Almost all people with this condition either smoke cigarettes, chew tobacco, or use other forms of tobacco^[1][2].

Scientists believe that something in tobacco damages the lining of blood vessels. The disease may involve problems with the immune system and how the body responds to tobacco. Some research suggests it could be related to tobacco hypersensitivity or that it may trigger an immune response in people who are susceptible to it^[2][3].

There may also be a genetic link to the disease. People with thromboangiitis obliterans have been found to have increased levels of certain immune system markers, suggesting that genes may make some people more likely to develop the condition when they use tobacco^[3][4].

Risk factors for developing thromboangiitis obliterans include smoking cigarettes, chewing tobacco, using marijuana, making your own cigarettes, being male, and being between the ages of 20 and 45^[2].

Symptoms and warning signs

The symptoms of thromboangiitis obliterans develop slowly over time. Early signs include severe pain in the legs and arms, which can happen when the body is at rest^[2].

Common symptoms include hand or foot pain that feels like burning or tingling. People may experience pain in the ankles, feet, or legs when walking, a condition called intermittent claudication. The pain is often located in the arch of the foot. As the disease gets worse, the pain may become constant and occur even at night while lying in bed^[1][5].

Other symptoms include fingers or toes that appear pale, red, or bluish and feel cold to the touch. The skin may turn white and then blue when exposed to cold, a condition known as Raynaud phenomenon. Depending on your natural skin color, these color changes may be harder or easier to see^[1][2].

People with this disease often develop sores or painful ulcers on their fingers or toes. These skin ulcers do not heal well. The affected hands or feet may feel cold and numb. Other signs include changes in skin color or texture, muscle cramps, and reduced hair growth in the affected areas^[2][5].

In some cases, inflammation in the veins can occur, moving from one location to another. This is called migratory superficial phlebitis and is present in up to 16% of patients. Occasionally, arthritis in the wrists or knees may develop before the blood vessels become blocked^[3][5].

Possible complications

The most serious complication of thromboangiitis obliterans is gangrene, which is the death of tissue due to lack of blood supply. When gangrene develops, amputation of fingers or toes may become necessary. If tissue damage becomes very severe and infected, surgery to remove the affected parts may be the only option^[1][5].

In people who continue to smoke, 43% require one or more amputations within about 7 to 8 years of diagnosis. The disease can keep getting worse even if you only smoke one cigarette each day^[2][3].

Rare complications include stroke, heart attack, transient ischemic attack (a temporary blockage of blood flow to the brain), problems with blood vessels in the intestines, and issues with the nervous system. Loss of blood flow in the entire limb of the affected fingers or toes can also occur^[2][5].

How is it diagnosed?

There is no single test that can diagnose thromboangiitis obliterans. Doctors make the diagnosis by considering several factors together and by ruling out other conditions that can cause similar symptoms^[8].

Your doctor will perform a physical examination and review your medical history, paying special attention to your tobacco use. During the exam, the doctor will check the pulses in your affected limbs, which are often weak or absent. Your hands, feet, fingers, or toes may appear pale or gray when raised above the heart and turn red or darker when lowered^[2][5].

Several tests may be used to examine your blood vessels. Ultrasound testing can detect decreased blood flow in the affected areas. An ankle-brachial index (ABI) test compares blood pressure in your ankle to blood pressure in your arm. More detailed imaging tests like computed tomography (CT) scans, magnetic resonance angiography (MRA), or catheter-based angiography can show blockages in the blood vessels^[2][8].

Blood tests are done to rule out other conditions such as diabetes, blood-clotting disorders, and autoimmune diseases like scleroderma or lupus. There are no blood tests that can directly diagnose thromboangiitis obliterans. An echocardiogram (heart ultrasound) may be done to look for blood clots coming from the heart. In rare cases when the diagnosis is unclear, a small tissue sample from the affected blood vessel may be removed and examined under a microscope^[5][8].

Doctors typically look for several key features when making a diagnosis: the person is usually younger than 50 years old, has a history of tobacco use, shows signs of blocked arteries in the hands or feet, and has no other explanation for the blockages^[4][5].

Treatment options

There is no cure for thromboangiitis obliterans. However, treatments can help control symptoms and prevent the disease from getting worse^[2][5].

The most important part of treatment is stopping all use of tobacco immediately. This is the only proven way to stop the disease from progressing. You must quit all forms of tobacco, including cigarettes, chewing tobacco, electronic cigarettes, vaping, and marijuana. Even using nicotine replacement products may keep the disease active. Avoiding secondhand smoke from other people is also important^[1][8].

When people with thromboangiitis obliterans stop smoking completely, their symptoms usually improve. In some cases, the disease goes into remission, meaning it becomes inactive. People who continue to smoke face a much higher risk of needing amputation^[2][3].

Several medications can help manage symptoms. Vasodilators, such as calcium channel blockers, are medicines that help open blood vessels and improve blood flow. Aspirin and other medicines may be prescribed to help prevent blood clots. Pain medications, including nonsteroidal anti-inflammatory drugs and stronger pain relievers, can help control ischemic pain^[2][11].

A medication called iloprost, which is a type of prostacyclin analogue, has shown benefit in some studies. It can help improve ulcer healing and reduce rest pain, although it is expensive and not available in all countries^[11][12].

In very severe cases, surgery may be considered. A procedure called surgical sympathectomy, which involves cutting nerves to the affected area, can help control pain. Bypass surgery to redirect blood flow around blocked vessels is rarely possible because the small vessels affected by this disease are usually not suitable targets for this type of surgery^[3][5].

Some newer treatments show promise. Spinal cord stimulation, which uses electrical signals to reduce pain, has been tried. Research into using growth factors like VEGF (vascular endothelial growth factor) and stem cell therapy has shown some positive results in helping to restore blood flow and prevent amputations^[11][16].

Taking care of your hands and feet is essential. Keep them warm and avoid exposure to cold temperatures, which can make blood vessels narrow. Treat any cuts or scrapes immediately, as poor blood flow makes healing slow. Wear comfortable shoes that don’t rub, and avoid tight socks that can restrict circulation. Apply warmth and do gentle exercises to help increase blood flow. If you develop a sore on your hand or foot, keep it dry and covered with a non-stick bandage until you see your doctor^[5][17].

What to expect

The outlook for people with thromboangiitis obliterans depends greatly on whether they stop using tobacco. Death from this disease is rare, but it can cause significant disability^[3].

If you quit all tobacco use immediately and completely, the disease can often be controlled, and amputation can usually be avoided. In people who stop smoking, disease remission is impressive, and the chance of avoiding amputation is much higher^[3][6].

In contrast, people who continue to use tobacco face a poor outlook. The disease will relentlessly worsen, and amputation becomes likely. Those who keep smoking may need repeated amputations over time^[5][6].

You should contact your doctor if you have symptoms of thromboangiitis obliterans, if your symptoms get worse even with treatment, or if you develop new symptoms^[5].