Buerger's disease

Not to be confused with Berger's disease (IgA nephropathy)

Buerger'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,^[1] primarily from smoking, but also from smokeless tobacco.

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Features

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.

Diagnosis

A concrete diagnosis of thromboangiitis obliterans is often difficult as it relies heavily on exclusion of the 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]

1. Age younger than 45 years
2. Current (or recent) history of tobacco use
3. Presence of distal extremity ischemia (indicated by claudication, pain at rest, ischemic ulcers or gangrene) documented by noninvasive vascular testing such as ultrasound
4. Exclusion of autoimmune diseases, hypercoagulable states, and diabetes mellitus by laboratory tests.
5. Exclusion of a proximal source of emboli by echocardiography and arteriography
6. Consistent arteriographic findings in the clinically involved and noninvolved limbs.

Pathophysiology

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.

Treatment

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.

Streptokinase has been proposed as adjuvant therapy in some cases.^[3]

Prognosis

Amputation is common and more severe in patients who continue to use tobacco. It often leads to vascular insufficiency. Buerger's is rarely immediately fatal, but rather a life shortening disease.

Prevention

The cause of the disease is unknown but heavily linked to tobacco use. There have also been links to persons with digestive disorders.

Epidemiology

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, who often smoke cigarettes made of raw tobacco (bidis).

History

Buerger's disease was first reported by Felix von Winiwarter in 1879 in Germany. ^[4]

It was described in detail by Leo Buerger in 1908 in New York,^[5] who called it presenile spontaneous gangrene after studying amputations in 11 patients.

References