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Introduction
- Buerger’s disease also called thromboangiitis obliterans is a chronic progressive disease of blood vessels. It mainly affects the blood vessels of the lower and upper limbs. Due to chronic inflammation, the blood vessels become narrower leading to a decreased blood supply to the affected limbs. When this decreased blood supply cannot meet the oxygen requirement of the limbs it causes pain.
- The lower limbs are usually affected more than the upper limbs. In 10% of cases, it can be bilateral.
- Most commonly affects people who are heavy smokers due to its strong association with tobacco in any form.
- Due to inflammation, the blood vessels can swell up to create a reduced inner diameter. It ultimately creates more thrombo-embolic potential. The inflammation spread and combined with thromboembolism and progressive narrowing, the affected limb may be gangrenous.
- Buerger’s disease is still poorly understood and hence can be challenging to treat.
Causes of Buerger's disease
- The exact causative factors for Buerger’s disease are yet to be discovered.
- Tobacco: It is believed that tobacco plays an important role in the onset of the disease and disease progression but the exact relationship between tobacco products and Buerger’s disease is not completely understood. Chemicals such as nicotine present in the tobacco cause irritation to the blood vessels making it swell. As a result, there is a decreased blood flow to the vessels. Due to this reduced blood supply, tissues don’t receive enough oxygen and nutrients that they need to survive and eventually develop ischemia, leading to ulcers, gangrene, and severe pain.
- Auto-immune: Tobacco may also trigger the immune response to incite inflammatory changes.
- Genetic predisposition: Some people may have multiple genetic factors that make them more susceptible to develop Buerger’s disease.
Risk factors
- Smoking or chewing tobacco
- Age: Younger age 20–30 years (under 45 years); with a heavy smoking habit or chewing tobacco. People above 50 years of age are also vulnerable to this disease due to an increased rate of smoking.
- Sex: Male is more affected than females. In recent years, women are being more affected than before because of an increase in the number of women smokers.
- Region: It most commonly affects those from Southeast Asia, India, and the Middle East, but rare among African–Americans, Europe, and the United States.
- Diabetes Mellitus
- Hyperlipidemia/hypertension
- Poor hygiene
- Recurrent minor foot injuries
- Low socio-economic group
Clinical features of Buerger's disease
- Cramping pain in the feet and/or hands during exercise/walking that may come and go (Claudication)
- Rest pain in the fingers or toes
- Numbness or tingling sensation in the limbs
- Skin becomes thin and shiny
- Diminished hair growth or loss of hair in the affected part
- Thick nails
- Temperature change in the affected area
- Color changes- distal extremities become pale/blue on exposure to cold (Raynaud’s phenomenon)
- Skin ulceration
- Gangrene of fingers or toes
Diagnosis
Diagnosis is made clinically by proper history and examination.
- History
- Age, sex, smoking habit history
- Presence of claudication and rest pain etc.
- Physical examination of the peripheral vascular system
- General inspection to see any skin changes, loss of hair, ulcer or gangrene
- Change of temperature
- Capillary refill time (if delayed more than 2s, this indicates arterial disease)
- Auscultation to check bruits
- Peripheral pulses
- Wrist and ankle pulses are absent but brachial and popliteal pulses are usually present
- Special tests
- Allen’s test to check the patency of the ulnar and radial arteries in the wrist
The patient will clench his/her fist, and then pressure is applied to the arteries in the wrist for several seconds. This will stop the blood flow and the hand will become cool and pale. The patient will extend his/her fingers, palm up, which should show a “blanched” hand. The doctor then releases the pressure on one of the arteries (either ulnar or radial artery near the wrist) and the hand is observed for “blushing”. If the color of the hand does not return within 5-10 seconds and remains pale and cold, the Allen test is considered to be negative or abnormal which means one artery is not enough to supply blood to his/her hand and fingers. If the hand quickly becomes warm and returns to normal color, this means positive or normal Allen test.
- Buerger’s test to check the patency of the arteries of the lower limbs
Lifting both legs to 45 degrees above the horizontal (Buerger’s angle), supporting the heels which leads to the development of pallor. Buerger’s sign is the progressive change in color from white to pink while return to a dependent position. If instead of being pink the limbs become flushed red (reactive hyperemia), this indicates severe disease.
Diagnostic criteria
Several different criteria have been proposed for the diagnosis of Buerger’s disease
- Currently, two different diagnostic criteria are accepted. Shinoya (1998) and Olin (2000) established these criteria.
Investigations
- There is no specific test available to confirm the diagnosis for Buerger’s disease
- Routine blood tests are done to rule out other conditions that may cause similar signs and symptoms
- An angiogram of the upper and lower extremities
- Shows “corkscrew” appearance of arteries due to vascular damage as a result of lack of blood flow, particularly in the wrists and ankles
- Skin biopsies of affected limbs are rarely performed because the biopsy site may not heal well due to poor blood supply
Differential diagnosis
- Atherosclerosis
- Endocarditis
- Raynaud’s phenomenon associated with connective tissue disorders (e.g. lupus or scleroderma)
- Diabetic foot
- Substance abuse (such as marijuana)
Treatment
Conservative management
- Stop smoking
- Currently, there is no specific treatment available for Buerger’s disease. Quitting all tobacco-containing products immediately and completely is the most effective way to arrest the disease from getting worse but it will not cure the disease, only prevent its progression.
- Doctors can counsel and recommend non-nicotine products or medications that will help to stop smoking.
- Another way to help deal with the cravings for cigarettes is by attending residential smoking cessation programs. Here the patient stays at a treatment facility for a number of days or weeks, during which he/she participates in daily counseling sessions and other activities to learn to live a tobacco-free life.
- Intermittent compression of the arms and legs to increase blood flow
- Moderate aerobic activity, such as walking or biking for at least 30 minutes to improve circulation
- Specific exercises intended to improve circulation to the feet and legs
- Skincare to prevent infection/gangrene. Keep skin protected from cold exposure
- Drink plenty of water and stay active to increase blood circulation
- Spinal cord stimulation
- A device known as spinal cord stimulator (SCS) is implanted under the skin that sends a low level of electrical current directly to the spinal cord to relieve pain.
Medications:
- Symptomatic therapy includes drugs
- To relief pain such as vasodilators like prostacyclin and calcium antagonists
- To prevent the blood from clotting (anticoagulants)
- Therapeutic angiogenesis (medications to stimulate the growth of new blood vessels)
- Topical ointments or antiseptics to heal ulcers
Surgical interventions:
- Surgical sympathectomy to cut the nerves to the affected area in order to control pain and increase blood flow may also be helpful.
- Major limb amputation is the most frequent outcome if patients continue to smoke.
Prognosis
- Death from Buerger’s disease is rare
- Buerger’s disease, despite being a severe form of vasculitis, does not involve other major organs such as the lung, kidneys, brain, and gastrointestinal tract, unlike many other forms of vasculitis.
- It is not fatal but if left untreated for a long time, it may lead to severe infection and gangrene which may require limb amputation.
Prevention
- Avoid smoking and all forms of tobacco
- Avoid stressful situation
- Consult a doctor and seek medical treatment as soon as possible if symptoms start to appear before letting it get worse
- Dietary modification- reduce fat and cholesterol intake.
- Avoid cold exposure and wear proper footwear to prevent foot ulceration.
