Buerger's disease
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Not to be confused with Berger's disease (IgA nephropathy) Buergers's disease (also known as thromboangiitis obliterans) is an acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet. It is strongly associated with use of tobacco products, primarily from smoking, but also from smokeless tobacco.
 

There is a recurrent acute and chronic inflammation and thrombosis of arteries and veins of the hands and feet. The main symptom is pain in the affected areas. Ulcerations and gangrene in the extremities are common complications, often resulting in the need for amputation of the involved extremity.
 

This disease was first reported by 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.
 

A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of other conditions. The commonly followed diagnostic criteria are below although the criteria tend to differ slightly from author to author. Olin (2000) proposes the following criteria:[2]
 

-Typically between 20-40 years old and male, although recently females have been diagnosed.
 

-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 such as ultrasound
 

-Exclusion of other 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.
 

 

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 no treatment known to be effective.
 

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. 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.
 

There are characteristic pathologic findings of acute inflammation and thrombosis (clotting) of arteries and veins of the hands and feet (the lower limbs being more common). The mechanisms underlying Buerger's disease are still largely unknown. It is suspected that immunological reactions play a role.
 

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.
 

Symptoms are treated as there is no treatment for the disease. Cessation of tobacco use may slow any further progression of the disease. Vascular surgery can sometimes be helpful in treating limbs with poor perfusion secondary to this disease. Use of vascular growth factor and stem cell injections have been showing promise in clinical studies.
 

Buerger's is not immediately fatal, but life-shortening. Amputation is common and major amputations (of limbs rather than fingers/ toes) are almost twice as common in patients who continue to smoke. Death rate has not been consistently shown as higher in patients who do not cease smoking but for this and other health concerns quitting is highly recommended. Female patients tend to show much higher longevity rates than men.
 

Despite the clear presence of inflammation in this disorder, anti-inflammatory agents such as steroids have not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low dosage intermittant form. Similarly, strategies of anticoagulation (thinning of the blood with aspirin or other agents to prevent clots) have not proven effective. The only way to slow the progression of the disease is to abstain from all tobacco products.
 

The cause of the disease is unknown but thought to be autoimmune in nature and heavily linked to tobacco use. There have also been links to persons with digestive disorders.
 

Buerger's is more common among men than women. It is more common in Israel, Japan and India along the "old silk route" than in the United States and Europe. The disease is most common among South Asians.

 

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