Buerger's disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Hadeel Maksoud M.D.[2]
Overview
Buerger's disease is an acute inflammation and thrombosis of the 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. It is more often seen in men. There may be an autoimmune element as well. Raynaud’s phenomenon, ulcers and pain are typically seen. Immediate termination of smoking is essential.
Historical Perspective
Buerger's disease was first discovered by Felix Von Winiwater, a German physician, in 1879. It was described in detail by Leo Buerger in 1908 in New York, who called it presenile spontaneous gangrene after studying amputations in 11 patients.
Classification
Buerger's disease may be classified according to the type and size of corkscrew collaterals present into 4 subtypes: type I, artery diameter >2 mm, large helical sign; type II, diameter >1.5 mm and ≤2 mm, medium helical sign; type III, diameter ≥1 mm and ≤1.5 mm, small helical sign; and type IV, diameter <1 mm, tiny helical sign.
Pathophysiology
Buerger's disease or thromboangiitis obliterans is a segmental vascular disease that causes occlusion and inflammation of the small and medium-sized vessels. Buerger's disease vasculitis is unique in having features of hypercellularity with intraluminal thrombi in the vessel wall, but sparing the elastic internal laminae of the vessel wall. The details of pathogenesis is still largely unknown.
Causes
Buerger's disease may be caused by tobacco smoking, Rickettsial infection, or autoimmune phenomena.
Differentiating Buerger's disease from Other Diseases
Buerger's disease must be differentiated from other diseases that cause ischemic vasculitis such as atherosclerosis, raynaud phenomenon, thromboembolic disease, repeated trauma, vasculitides, frostbite, scleroderma, and systemic lupus erythematosus.
Epidemiology and Demographics
Buerger disease 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). Buerger's disease affects more males than females at a ratio of 3:1. The prevalence in the United States has decreased from 140 per 100,000 in 1947 to 8 - 12 per 100,000 as the prevalence of smoking has declined.
Risk Factors
The most potent risk factor in the development of Buerger's disease is tobacco smoking. Other risk factors include male sex, rickettsial infection, South Asian or Middle Eastern descent, age between 20-45 years and a medical history of Raynaud's disease or autoimmune disease.
Screening
There is insufficient evidence to recommend routine screening for Buerger disease.
Natural History, Complications, and Prognosis
If left untreated, 75% of patients with Buerger's disease may have a relapsing and remitting course, whilst 20% may progress and 5% may have a benign course of Buerger's disease. Common complications of Buerger disease include amputation, gangrene and loss of circulation beyond the affected hand or foot. Smoking cessation leads to an 8-fold decrease in the risk for amputation. Amputation is common and more severe in patients who continue to use tobacco, which often leads to vascular insufficiency. Buerger's disease is rarely immediately fatal, but rather a life shortening disease. Prognosis of Buerger's disease varies from person to person, depending on the patient's life-style and the severity of the damaged vessels.
Diagnosis
Diagnostic Study of Choice
Although clinical examination is sufficient for diagnosis, in cases where diagnosis is not definitive, a catheter-based arteriogram is the gold standard test for the diagnosis of Buerger disease. The following result of catheter-based arteriogram is confirmatory of Buerger disease and includes, absence of atherosclerosis, no cause for thromboembolism, small and medium-sized vessels involved, namely tibial, popliteal, and radial arteries, segmental affection of vessels between normal appearing segments and corkscrew collaterals described as collateralizations around an occlusion area but are not pathognomonic.
History and Symptoms
The hallmark of Buerger's disease is pain and/or ischemia of the digits. A positive history of history of tobacco smoking is suggestive of Buerger's disease. The most common symptoms of Buerger's disease include pain and pallor of the extremities with weakness and swelling of the affected limb. Less common symptoms of Buerger's disease include migratory phlebitis and claudication.
Physical Examination
Common physical examination findings of Buerger's disease include pallor, edema, swelling, ulceration and gangrene of the distal extremities.
Laboratory Findings
There are no diagnostic laboratory findings associated with Buerger's disease. Lab testing is done to exclude other vasculitides, such as lupus, scleroderma and mixed connective tissue disease.
Electrocardiogram
There are no ECG findings associated with Buerger's disease. ECG may detect atrial fibrillation, which may possibly be a source of thromboembolism and therefore, exclude Buerger's disease.
X-ray
There are no x-ray findings associated with Buerger's disease.
Echocardiography and Ultrasound
Color duplex ultrasound may be helpful in the diagnosis of Buerger's disease. Findings on an ultrasound suggestive of Buerger's disease include corkscrew collaterals. Echocardiography may be helpful in detecting the source of thromboembolism and therefore, exclude Buerger's disease.
CT scan
Multidetector computed tomographic angiography (MCTA) may be helpful in the diagnosis of Buerger's disease, as it is able to visualize vessels. However, MCTA lacks spatial resolution in visualizing the vessels of the hands and feets, where pathology is commonly found in Buerger's disease.
MRI
Gadolinium-enhanced magnetic resonance angiography (MRA) may be helpful in the diagnosis of Buerger's disease, as it is able to visualize vessels. However, MRA lacks spatial resolution in visualizing the vessels of the hands and feets, where pathology is commonly found in Buerger's disease.
Other Imaging Findings
Buerger's disease is diagnosed mostly clinically, however, in cases where diagnosis is indefinite and the extent of disease is unknown, a catheter-based arteriogram may be helpful in the diagnosis of Buerger's disease. Findings on a catheter-based arteriogram suggestive of Buerger's disease include the absence of atherosclerosis, lack of a source for thromboembolism, small and medium-sized vessel involvement, segmental affection of vessels between normal appearing segments, and corkscrew collaterals described as collateralizations around an occlusion area, however, this finding is not pathognomonic.
Other Diagnostic Studies
Biopsy is not routinely performed, however, may be carried out in patients older than 45 years presenting with nodules subcutaneously or migratory thrombophlebitis, with uncharacteristic large artery involvement and/or the presence of higher than normal anticardiolipin antibodies. Biopsy may therefore be helpful in the diagnosis of Buerger's disease. Findings on a biopsy suggestive of Buerger's disease include small and medium vessel involvement, hypercellularity with leukocytic infiltration, segmental affection, occlusion with inflammatory thrombi (may or may not be organized depending on stage of disease), sparing of the internal elastic laminae, formation of microabscesses, and multinucleated giant cells.
Treatment
Medical Therapy
There is no treatment for Buerger's disease. In order to prevent progression and control symptoms smoking cessation is crucial. Smoking cessation does not reverse damage already caused. Pharmacologic medical therapies for Buerger's disease include smoking cessation, palliative treatments, prostaglandin analogs and phosphodiesterase inhibitors, calcium channel blockers, endothelin receptor antagonists, compression therapy and some experimental therapies. It should be noted, however, that these therapies are purely palliative and do not reverse previous damage caused. Analgesics including NSAIDs and acetaminophen may be used to provide pain relief.
Surgery
Surgery is usually not feasible in Buerger's disease since the integrity of the distal vessels usually does not allow for revascularization, nevertheless, surgical intervention may be considered in order to maintain peripheral blood flow as much possible. Surgical intervention may also be carried out in order to provide pain relief, heal ulcers and decrease the chances of future amputations, such as sympathectomy.
Primary Prevention
An effective measure for the primary prevention of Buerger's disease includes smoking cessation. This should include complete abstinence without the use of nicotine-containing anti-smoking aids, such as transdermal patches and gum. However, the use of bupropion and varenicline as a means of preventing cravings is permissible.
Secondary Prevention
Effective measures for the secondary prevention of the complications of Buerger's disease include debridement and dressing of ulcers, wearing protective footwear to avoid injury and lack of healing thereafter, early detection and treatment of injuries in the extremities including the use of prophylactic antibiotics, wearing thick gloves and avoidance of cold weather, and finally, the use of vasoconstricting drugs must be avoided.