Buerger's Disease

Synonyms: Thromboangiitis obliterans
This is a disease characterised by inflammation and thrombosis of the small and medium size arteries and veins of the hands and feet.

The incidence is falling as the association with tobacco is recognised. In the USA it has fallen from around 100 per 100,000 people to less than 20 per 100,000. Women used to represent about 5% of cases but now they are about a third, reflecting smoking habits. It affects mostly men between the ages of 20 and 45. Recently it has been seen more in people over the age of 50.

There is considerable difference between races. It is more common in those of oriental race, including those from South-east Asia, India and the Middle East but it is less common in those of African origin. It is less common in people of northern European descent. Natives of India, Korea, and Japan, and especially Israeli Jews of Ashkenazi descent, have the highest incidence of the disease. Part of this difference in disease incidence may be due to variability in diagnostic criteria.¹
Rarely it affects children with auto-immune phenomena and a strong level of inflammation.

The association of Buerger's Disease with tobacco use, particularly cigarette smoking, cannot be overemphasized.
Most patients with Buerger's disease are heavy smokers, but some cases occur in patients who smoke "moderately". It has been postulated that Buerger's Disease is an "autoimmune" reaction triggered by some constituent of tobacco.²

Symptoms

The early symptoms of Buerger's Disease include claudication in the feet and/or hands, or pain in these areas at rest. The pain typically begins in the extremities but may radiate to more central parts of the body. Other signs and symptoms may include numbness and/or tingling in the limbs and Raynaud's phenomenon.

• Pain or tenderness can be very intense and often at rest
• Numbness and tingling in the limbs
• Symptoms that may worsen with exposure to cold or emotional stress
• Two or more limbs are affected
• Pain may increase with activity such as walking and decrease with rest
• Symptoms may worsen with exposure to cold or with emotional stress

Signs

• Skin ulcerations and gangrene of the digits are common
• Discoloration
• Later symptoms include enlarged, red, tender cord-like veins
• Pulses may be decreased or absent in the affected extremity

In the early stages other causes of Raynaud's phenomenon must be considered including SLE and scleroderma but the aggressive nature of Buerger's disease shows the true diagnosis as gangrene and ulceration set in. Exclude autoimmune diseases, hypercoagulable states and diabetes mellitus. A proximal source of embolism may be sought by echocardiography and arteriography.

Diagnosis based on a system of scoring points has been suggested³ although some find this a little cumbersome and difficult.

• Smoking history
• Onset before the age of 50 years
• Infrapopliteal arterial occlusions
• Either upper limb involvement or phlebitis migrans
• Absence of atherosclerotic risk factors other than smoking

Confident clinical diagnosis of Buerger's disease requires all 5 features. Strict clinical diagnostic criteria are essential for any study of a disease to ensure the homogeneity of the selected patient population for valid comparisons.

There are no specific serological markers to diagnose Buerger's disease.

• Recommended tests to rule out other causes of vasculitis include FBC, LFTs, creatinine, fasting glucose, ESR or PV, antinuclear antibody, rheumatoid factor, serologic markers for CREST syndrome and scleroderma, and screening for hypercoagulability.
• Angiography - Certain angiographic features are typical of Buerger's disease. These include a "corkscrew" appearance of arteries that results from vascular damage, particularly the arteries in the region of the wrists and ankles. Angiograms may also show occlusions or stenoses in multiple areas of both the arms and legs. It is sometimes necessary to perform angiograms of other parts of the body regions, like a mesenteric angiogram to exclude other forms of vasculitis that involve vascular regions atypical for Buerger's.
• A Doppler ultrasound may be helpful.
• Skin biopsies of affected extremities are rarely performed because of fear that a biopsy site in an ischaemic area will not heal.
• Echocardiography may be required to exclude a source of recurrent emboli

Patients with Buerger's disease must stop smoking immediately and completely. This is the only treatment known to be effective.
Supportive measures include:

• Gentle massage and warmth to increase circulation
• Avoid conditions that reduce peripheral circulation, like cold temperatures
• Avoid sitting or standing in one position for long periods
• Do not walk barefoot to avoid injury
• Avoid tight or restrictive clothing
• Energetic treatment of any injuries (ulcers etc.)

The only way to prevent the progression of the disease is to abstain from all tobacco products. Avoid vasoconstricting drugs.

Drug Treatment

The drug iloprost, a prostacyclin analogue has been shown to be superior to low dose aspirin.^5,6

Calcium channel blockers, steroids, anticoagulants and other antiplatelet drugs are ineffective.

If the disease is due to sensitivity to a component of tobacco other than nicotine then NRT may be used but prudence dictates that it should be continued for as short a time as possible.

Surgical

Sympathectomy may provide temporary relief but outcome is variable.⁷ Patients who continue to smoke are likely to require amputation of fingers and toes. Areas with gangrene must be removed surgically.

Ulcers, infection and gangrene must be treated energetically.

The disease is progressive in patients who do not stop smoking.

The first reported case was by von Winiwarter from Germany in1879 in an article entitled "A strange form of endarteritis and endophlebitis with gangrene of the feet." In 1908, Leo Buerger published a detailed description of the disease⁸ in which he referred to "presenile spontaneous gangrene." The paper discussed the pathological findings in 11 limbs amputated from Jewish patients in New York.

Leo Buerger was born in Vienna in 1879 but his parents emigrated to New York the following year. He studied at the College of Physicians and Surgeons, Columbia University, where he graduated in 1901. He studied at the Breslau Surgical Clinic in Germany for a time and then returned to private practice in New York where from 1905 he had an attachment to the Mount Sinai Hospital as professor of urological surgery.