- Definition: Idiopathic non-necrotizing panarteritis.
- Demographics: 98% male, 99% smokers
- Imaging findings: Segmental vascular occlusions, corkscrew vessels, large vessels will appear normal
- Distribution of disease: Legs & feet | forearms & hands
- Treatment: stop smoking. This may even reverse disease.
- Differential diagnosis: lupus, scleroderma, drug abuse
Buerger’s disease, also known as thromboangiitis obliterans, is a rare autoimmune disorder that causes inflammation and blood clotting in the small and medium-sized arteries and veins of the arms and legs. The disease is most common in young men who smoke tobacco, and can lead to serious complications, such as tissue death and amputation.
Symptoms of Buerger’s disease typically develop gradually, beginning with pain, numbness, and tingling in the extremities. As the disease progresses, individuals may experience difficulty walking, ulcers on the skin, and gangrene, or tissue death, in the affected extremities.
The cause of Buerger’s disease is not fully understood, but it is thought to be an autoimmune disorder in which the body’s immune system mistakenly attacks healthy tissue. This leads to inflammation and blood clotting in the arteries and veins, which can cause them to narrow or become blocked.
Diagnosis of Buerger’s disease is typically made based on a combination of clinical symptoms, imaging tests such as angiography, and blood tests. Treatment for the disease typically involves quitting smoking, as well as the use of medications to control inflammation and prevent further damage to the blood vessels.
While there is no cure for Buerger’s disease, early diagnosis and treatment can help prevent serious complications, such as tissue death and amputation. It is important for individuals who are at risk for Buerger’s disease, particularly young male smokers, to be monitored by a healthcare provider and receive regular check-ups to ensure the condition is properly managed.
SUMMARY:
●Thromboangiitis obliterans (TAO) is an inflammatory vascular disease of unclear etiology that affects medium and small arteries, veins, and nerves in the extremities, with rare involvement of visceral and cerebral vessels. Patients are predominantly young smokers who present with pain from distal extremity ischemia, ischemic digital ulcers, or digital gangrene. The disease is strongly associated with the use of tobacco products and also with cannabis.
●TAO is characterized by segmental inflammatory thrombotic vessel occlusions. It is distinguished pathologically from other forms of vasculitis by a highly cellular, inflammatory intraluminal thrombus but no fibrinoid necrosis and the presence of an intact internal elastic membrane. Although smoking is important to the pathogenesis, the specific etiologic mechanism remains unknown. Inflammatory, immunologic, and endothelial factors play a role.
●The evaluation of TAO consists of history and physical examination, vascular evaluation, and laboratory examination to serologically rule out other etiologies that can cause small and medium vessel occlusive arterial disease.
●A clinical diagnosis without the need for biopsy can be established with the following commonly used criteria
•Age less than 45 years
•Current or recent history of tobacco use
•Distal extremity ischemia (objectively noted on vascular testing)
•Typical arteriographic findings of thromboangiitis obliterans
•Exclusion of autoimmune disease, thrombophilia, diabetes, and proximal embolic sources
●Imaging studies are not necessary to establish a clinical diagnosis but may be helpful in patients whose clinical features are not definitive. When imaging is performed, features suggestive of thromboangiitis obliterans include lack of atherosclerosis, no proximal source for embolism, the involvement of small and medium-sized vessels, segmental vascular occlusion, and collateralization around areas of occlusion (ie, corkscrew collaterals). However, such findings are nonspecific since they may be found in patients with other causes of small vessel occlusive disease.
●Biopsy of a subcutaneous nodule or vein with superficial thrombophlebitis that shows typical acute phase changes on histology provides a definitive diagnosis, but biopsy is not usually needed.
●For patients who smoke, we recommend smoking cessation. In addition to other demonstrated benefits, smoking cessation reduces symptoms and decreases the risk for major amputation. Continued smoking is associated with an overall amputation rate of 40 to 50 percent.
●For patients with ischemic pain, digit ulceration, or gangrene, intermittent pneumatic compression may improve pain by increasing blood flow. Calcium channel blockers may also help reduce vasospasm associated with Raynaud phenomenon. Other treatments that may be considered to manage pain include intravenous prostaglandin infusion, and possibly phosphodiesterase inhibitors.
●Revascularization is usually not indicated due to the distal nature of occlusive disease and because most patients do well with smoking cessation. Bypass surgery may be considered in select patients with severe ischemia and suitable distal target vessels. Although the long-term patency rates are inferior to those seen with peripheral artery disease due to atherosclerosis, limb salvage rates are generally greater than 90 percent, but may be more correlated to smoking cessation.
References:
Klein-Weigel PF, Richter JG. Thromboangiitis obliterans (Buerger’s disease). Vasa. 2014 Sep;43(5):337-46. doi: 10.1024/0301-1526/a000371. PMID: 25147010.
https://pubmed.ncbi.nlm.nih.gov/25147010/