What is Buerger’s Disease?

By Emily Henderson, B.Sc.

Buerger's disease, also known as thromboangiitis obliterans, is a rare condition that primarily affects young to middle-aged male smokers. The veins and arteries of the extremities constrict or block (occlusion), resulting in diminished blood flow to these areas (peripheral vascular disease). It is nonatherosclerotic inflammatory arthritis that affects the arteries and veins of the extremities.

Despite its significant link to tobacco use, little is known about the disease's overall pathogenesis. Legs are more commonly affected than arms. The most common symptom is severe discomfort in the lower arms and legs when lying down. Individuals with Buerger's disease may develop tissue death (gangrene) in affected limbs in extreme situations.

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Causes and symptoms

The actual cause of Buerger's disease is yet to be identified. Tobacco usage, on the other hand, has been linked to the development of the illness. Most researchers feel that using tobacco products in the past or present is a necessity for diagnosing Buerger's illness. The precise association between tobacco products and Buerger's illness is unknown.

Buerger's illness is thought to be an autoimmune disorder by some scientists. Trauma to the hands and feet may play a role in the disease in some circumstances. Because the prevalence of Buerger's illness varies greatly between ethnic groups, genetic factors may play a role in its development or severity.

Buerger's disease is defined by the constriction or occlusion (occlusion) of the intermediate or small arteries and veins of the extremities, resulting in decreased blood flow to these areas (peripheral vascular disease). Buerger's disease usually manifests itself in brief (acute) episodes that last from one to four weeks.

The condition has a recurring pattern. The most common symptom of Buerger's disease is severe pain in the lower arms or legs when lying down. Affected persons may also have cramps in their legs when walking, which may cause them to limp (claudication) in rare circumstances.

Sores (ulcers) on the arms and legs, numbness, tingling, pale discoloration of the hands, and a lack of normal blood flow to the fingers or toes when exposed to cold temperatures (Raynaud's phenomenon), and inflammation and clotting of certain veins are all physical symptoms associated with Buerger's disease (thrombophlebitis). Dry, black ulcerations on the tips of the fingers or toes can be very painful. Elevation may aggravate the pain associated with these ulcers. Individuals with Buerger's disease may develop tissue death (gangrene) in the afflicted areas in extreme cases.

The arteries and veins of the intestines may also be damaged in some circumstances. This can cause severe abdominal heaviness or pain (angina) as well as weight loss. Affected people have shown neurological problems in a very small percentage of instances (less than 2%).

Epidemiology

Buerger's disease is a very rare disease that mostly affects young or middle-aged male cigarette smokers who first develop symptoms before the age of 40-45. More affected females have been recorded in the medical literature in recent years. According to some scientists, this is related to an increase in the number of women who smoke.

Although the ratio of affected men to women was once 100:1, the subsequent medical literature has indicated that the ratio may now be closer to 10:1. Individuals who do not smoke have developed Buerger's disease in extremely uncommon circumstances. A rise in the prevalence of the condition in women has been observed in several studies, ranging from 11% to 23%.

In the United States and Europe, Buerger's disease is exceedingly rare, but it is more frequent in other regions of the world, particularly Asia and the Far and the Middle East. In the general population of the United States, the incidence is estimated to be 12.6-20 per 100,000 people.

The condition's prevalence among all patients with peripheral vascular disease ranges from 0.5 to 5.6 percent in Western Europe to 45 to 63 percent in India, 16 to 66 percent in Korea and Japan, and 80 percent among Jews of Ashkenazi heritage in Israel. Buerger's disease is more common in places where tobacco is heavily used.

Diagnosis and treatment

The identification of typical physical traits and symptoms can lead to a diagnosis of Buerger's disease. To diagnose Buerger's disease, many doctors require a history of recent or current cigarette usage. Tests including angiography or non-invasive procedures may be employed to confirm the diagnosis. During angiography, a specific dye is injected into the blood arteries to make them visible on x-rays.

Clinical and angiomorphologic criteria provided by Olin et al. and Shionoya are frequently used to make a diagnosis. The latter is easy to remember because it is based on only five factors. In around 20%–25% of instances, both the upper and lower extremities are involved. Buerger's disease cannot be diagnosed just on a single limb affliction. It does not have any particular biomarkers. Laboratory testing is still necessary to rule out other conditions such as diabetes, connective tissue disease, vasculitides, or congenital or acquired thrombophilia.

Buerger's disease is treated with symptomatic and supportive care. When people stop smoking, they may experience complete remission of their disease. Treatment options for Buerger's disease should avoid premature or needless surgery if an affected individual does not cease smoking. Anticoagulants, vasodilators, anti-inflammatories, antibiotics, or pain relievers may be used as conservative treatments (analgesics).

Surgery may be required in some circumstances. Nerve terminals (ganglia) are removed in affected persons during surgery to block the nerve pathway and enhance blood flow to the affected limb. Surgeons may use bypass techniques to get past blocked or narrowed (occluded) veins or arteries in some circumstances. Surgeons may be obliged to amputate a finger or toe, or a portion of an arm or leg, in severe circumstances.

The use of bone marrow-derived stem cells may help individuals with Buerger's disease heal ulcers and improve their pain-free walking distance, as per very low-quality research. High-quality trials evaluating the efficacy of stem cell therapy for the treatment of Buerger's disease patients are required.

Buerger's disease is an unidentified medical ailment that is intrinsically associated with cigarette consumption. Other therapeutic approaches are necessary because surgical revascularization is rarely possible, although few randomized clinical trials are testing their effectiveness. At present, complete abstinence from all tobacco use is the cornerstone of management.

References:

• (2021). Smoking and Buerger’s Disease. [Online] Centers for Disease Control and Prevention. Available at: https://www.cdc.gov/tobacco/campaign/tips/diseases/buergers-disease.html
• Le Joncour, A., Soudet, S., Dupont, A., Espitia, O., Koskas, F., Cluzel, P., Hatron, P. Y., Emmerich, J., Cacoub, P., Resche-Rigon, M., Lambert, M., Saadoun, D., & French Buerger's Network (2018). Long-Term Outcome and Prognostic Factors of Complications in Thromboangiitis Obliterans (Buerger's Disease): A Multicenter Study of 224 Patients. Journal of the American Heart Association, 7(23), e010677. https://doi.org/10.1161/JAHA.118.010677
• Cacione, D. G., do Carmo Novaes, F., & Moreno, D. H. (2018). Stem cell therapy for treatment of thromboangiitis obliterans (Buerger's disease). The Cochrane database of systematic reviews, 10(10), CD012794. https://doi.org/10.1002/14651858.CD012794.pub2
• Ignacio J. Rivera-Chavarría, José D. Brenes-Gutiérrez. (2016). Thromboangiitis obliterans (Buerger's disease). Annals of Medicine and Surgery, 7, Pages 79-82. https://doi.org/10.1016/j.amsu.2016.03.028.
• Klein-Weigel, P., Volz, T. S., Zange, L., & Richter, J. (2016). Buerger’s disease: providing integrated care. Journal of multidisciplinary healthcare, 9, 511.
• (2007). Buerger’s Disease. [Online] National Organization for Rare Disorders. Available at: https://rarediseases.org/rare-diseases/buergers-disease/

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