Thromboangiitis obliterans

Buerger's disease
Classification and external resources
Complete occlusion of the right and stenosis of the left femoral artery as seen in a case of thromboangiitis obliterans
ICD-10	I73.1
ICD-9	443.1
OMIM	211480
DiseasesDB	1762
MedlinePlus	000172
eMedicine	med/253
MeSH	C14.907.137.870

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

Thromboangiitis obliterans (also known as Buerger's disease) is a recurring progressive inflammation and thrombosis (clotting) of small and medium 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.

Signs and symptoms

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 Leo 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.

Diagnosis

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

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

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 stenosis (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 femoral artery distribution).

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.

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.

Most patients with Buerger’s are regular smokers, but some cases occur in patients who only 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.

Prevention

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

A possible role for Rickettsia in this disease has been proposed.

Treatment

Smoking cessation has shown to slow the progression of the disease and decrease the severity of amputation in most patients, but does not halt the progression.

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.

In addition Limaprost, an oral PGE1 derivative, has shown efficacy in one published randomized trial.

Prognosis

Buerger's is not immediately fatal, but it is 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 corticosteroids have not been shown to be beneficial in healing, but do have significant anti-inflammatory and pain relief qualities in low dosage intermittent 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.

Epidemiology

Buerger's is more common among men than women. It is more common in , Japan, India, and Manipur along the "old silk route" than in the United States and Europe. The disease is most common among South Asians. Incidence of Thromboangiitis obliterans is 8 to 12 per 100,000 adults in the United States (0.75% of all patients with peripheral arterial disease).

History

Buerger's disease was first reported by Felix von Winiwarter in 1879 in Austria. It wasn't until 1908, however, that the disease was given its first accurate pathological description, by Leo Buerger at Mount Sinai Hospital in New York City. Buerger called it "presenile spontaneous gangrene" after studying amputations in 11 patients.

As reported by Alan Michie in God Save The Queen, published in 1952 (see pages 194 and following), King George VI was diagnosed with the disease in late 1948 and early 1949. Both legs were affected, the right more seriously than the left. The King's doctors prescribed complete rest and electric treatment to stimulate circulation, but either unaware of the connection between the disease and smoking (the King was a heavy smoker) or unable to persuade the King to stop smoking, the disease failed to respond to their treatment. On March 12, 1949, the King underwent a lumbar sympathectomy, performed at Buckingham Palace by Dr. James R. Learmonth. The operation, as such, was successful, but the King was warned that it was a pallative, not a cure, and that there could be no assurance that the disease would not grow worse. From all accounts, the King continued to smoke.

References

• Aktoz T, Kaplan M, Yalcin O, Atakan IH, Inci O (December 2008). "Penile and scrotal involvement in Buerger's disease". Andrologia 40 (6): 401–3. doi:10.1111/j.1439-0272.2008.00859.x. PMID 19032693. http://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0303-4569&date=2008&volume=40&issue=6&spage=401. 

External links

• 00394 at CHORUS
• Buerger's disease at Who Named It?
• Brandon Carmichael
• Johns Hopkins University [1]