Buerger’s disease: Smokers beware

Buerger's disease, also known as thromboangiitis obliterans, is a rare disease of the small arteries and veins in the arms and legs. The disease is characterized by narrowing/blockage of the blood vessels, mainly in the hands and feet, leading to blockage of the blood vessel. This eventually leads to gangrene of the fingers or toes. Buerger's disease is rare in the United States, but is very common in the Middle East and Far East. Buerger's disease most commonly affects men between ages 20 and 40, though it's becoming more common in women. With the recent wave of immigrants, the disorder is now becoming more prominent in North America. The majority of individuals diagnosed with buergers disease smoke cigarettes or use 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 ultimately be necessary. The first reported case of thromboangiitis obliterans was described in Germany in 1879. A little more than a quarter of a century later, in Brooklyn, 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." The paper discussed the pathological findings in 11 limbs amputated from Jewish patients with the disease. It was then discovered that the disorder was intimately associated with smoking and affected the small blood vessels in the hands and the feet. The disorder typically involves the distal vessels with proximal progression and is most often seen in middle aged men between the ages of 20-40, who have a history of smoking. Even though usually seen in men, cases have been reported in women. While more common in Middle and Far Eastern races, the disease is seen universally in all races. While the exact cause of this disorder remains unknown, all patients with TAO are smokers. It is believed that TAO is an autoimmune disorder (your own immune system starts to attack your body), most likely triggered by the use of tobacco.

Buerger’s disease generally affects the small and medium sized vessels in the body. It may involve the blood vessels in the arms, hands legs and feet. In addition the blood vessels to the kidney, bowels and event the brain may be involved. When the blood vessels are affected, the vessel becomes narrower and eventually closes off-resulting in no blood supply. There are numerous reports that the small blood vessels supplying the penis are also blocked and the majority of these individuals also complain of impotence (erectile failure). While the etiology of Buerger disease is unknown, exposure to tobacco is essential for both initiation and progression of the disease. The idea 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. In the Middle East, Asia and the Pacific where tobacco use is endemic, the condition is extremely common. While the majority of the cases are seen in smokers, a few cases have also been reported in non smokers and this has been attributed to either the use of chewing tobacco or second hand smoke.

Buerger’s disease is relatively less common in both the North American and Northern European population. With the increasing awareness of the detrimental effects of smoking, the prevalence of Buerger disease has significantly decreased over the past decade. Fifty years ago, Buerger’s was found in 104 cases per 100,000 patients. Today, the disorder is seen in about 10-20 cases per 100,000 patients. In all series reported so far, Buerger’s disease is far more common in males than females by a ratio of 3:1. However, over the last 15 years, this ratio is decreasing as more women continue to smoke. The average age of patients who present with this disease is 20-40 years. Death is unlikely from Buerger’s disease but the morbidity is very high. The disorder is quite rare in populations who do not smoke but rampant in countries where smoking is a way of life. India, Saudi Arabia, Bangladesh and the Middle East have some of the highest incidence of Buerger’s disease, whereas Japan, Korea and Northern Europeans have the lowest. In North America, reports do indicate that with the recent immigration, the disease is being seen more frequently.

Buerger’s disease causes a severe inflammation of the small blood vessels. The vessels eventually block off. As they block off, the blood supply to the toes and fingers decreases. In addition, the nerves in the toes and fingers also die as a result of no oxygen. It is these two features which are responsible for the symptoms of Buerger’s disease. Although cigarette smoking is the chief cause of the disorder, other smoke related products are just as harmful. Cigars, chewing tobaccos and nicotine in any form have been associated with the disorder.

Most individual have symptoms before the age of 30-35 and the majority are men. The presentation is varied and ranges from pain in the fingers to frank gangrene. An additional complaint is extreme sensitivity to the cold. Numbness, burning or tingling in the extremities is common when the nerves start to die. Those who seek medical attention late in the course of their disease may present with foot infections and, occasionally, dead toes. All individuals with Buerger’s disease will have some of the following features: - severe pain in the legs, feet - severe pain in the fingers - weakness in the feet and arms - fingers and toes turning blue when exposed to cold - ulcers on the toes and fingers - gangrene of the toes and fingers When Buerger’s disease has started, the best thing is to quit smoking. If smoking is continued, the disease progresses and eventually leads to amputation of the toes or fingers and sometimes, even the limbs. Those with a lot of bad luck may even lose their erectile ability and others may develop a stroke and end up in a wheel chair or be bed ridden for the rest of their life.

The diagnosis of Buerger's disease is not difficult. It should always be suspected in any individual who smokes and presents with pain or ulcers in the toes or fingers. The patient may also complain of extreme sensitivity of the fingers to cold and there may even be bluish discoloration present. But there is no blood test that is used to make a diagnosis. The diagnosis is made on clinical features. Biopsy is only done to exclude other disorders such as lupus, diabetes or scleroderma. All blood tests in these individuals are normal. Some individuals may require an angiogram which may support the diagnosis of Buerger’s disease but does not confirm it. In Buerger’s disease, only the small and medium size blood vessels are affected- which is also a similar finding in diabetes and lupus. Some physicians feel that angiography should be the first diagnostic test because some arteriographic changes consistent with Buerger’s disease are frequently seen in limbs that are not yet clinically involved. In some cases, the diagnosis of Buerger’s is difficult and a number of criteria have been established to help make the diagnosis. The criteria include: a. Men or women usually between the ages of 20-40 b. Both past and recent history of smoking c. Presence of distal extremity ischemia d. History of claudication e. pain in the toes and fingers even at rest f. ulcers on the toes and fingers g. gangrene of the toes and fingers h. no evidence of lupus, diabetes or any collage vascular disorder i. no disease anywhere else in the body j. arteriography findings reveal narrowing and occlusion of the affected limbs

In a few individuals, additional studies may be required to determine the extent of the disease. An ultrasound can reveal the absence of blood flow and can even reveal the presence of blood clots in the veins.

- Ulceration of toes and fingers - Gangrene of the toes and fingers - Extreme pain - Possible loss of kidney and renal failure - Possible bowel ischemia - Possible stroke - Minor or major amputation

There is only treatment for Buerger’s disease: Quit using tobacco in any form. If one has been told he/she has Buerger’s disease, then tobacco use must be stopped immediately to prevent disease progression. Gradually weaning of cigarettes and nicotine patches are a complete waste of time. STOP SMOKING IMMEDIATELY. As soon as smoking is stopped, the vessels will gradually stop getting worse but the damage done can not be reversed- even if you pray faithfully. For those who whine about not being able to stop smoking, they should be prepared to have an amputation of their toes of finger, sooner or later. One should take a trip to a wound or vascular clinic and the results will be obvious- smoking sucks. Any other therapy provided is just supportive do mange the pain and prevent the infection from getting worse.

Recently, treatment with intravenous iloprost (a prostaglandin analogue), is suggested for Buerger’s disease. The treatment is expensive and may open up the blocked vessel and improve blood flow. However, this is not seen in everyone. The treatment is not curative but may provide a time delay before the eventual amputation. Various other products have recently become available to heal the ulcers. These growth factors and growth genes have to be applied to the ulcer. However, when there is no blood, nothing really helps.

When an ulcer has already occurred on the toe of finger, supportive care must be undertaken such as: - wearing protective shoes to prevent any further trauma - keep the wounds clean and dry to prevent infection - Warm garments to protect against cold sensitivity - avoid drugs that may worsen the disease - may take an aspirin everyday

The role of surgery in the treatment of Buerger’s disease is negligible. Since the disease affects the very small vessels in the extremities, surgery is unable to improve it. Most patient with Buerger’s who are admitted to the hospital have ulcers or gangrene of the digits. Every attempt is made to preserve the digits with minimal surgery. However, in those cases with frank gangrene, amputation of the digits is the only solution. Amputation is always the last resort but in many cases, the intense pain and dead digits offer no other alternative. In the very rare patient, there may be an isolated narrowing of a vessel which can be ballooned with angioplasty. Even if surgery is done, it is almost never successful and in fact makes the condition worse.

Individuals with Buerger’s have no dietary restrictions and should continue to exercise. Because Buerger’s is a progressive disease if the individual continues to smoke, continuous out patient follow up is required. Each visit includes evaluation of the pulses and extremities. Appropriate wound care is done during each visit. It is highly recommended that evaluation be done by a team of specialist. Almost the majority of these patients require pain medications and referral to pain control specialist is advised

How the individual does in future is strictly dependent on whether he smokes or not. The only treatment for Buerger’s disease is to stop smoking. For those who quit smoking, the majority avoid an amputation, but for those who continue smoking, there is nearly a 50% chance of an amputation. In addition, the disease continues and multiple amputations are the norm. Even after cessation of smoking, the individuals may have significant pain in their extremities and any open ulcer may have a prolonged healing phase.