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Obliterative thrombangiitis
Last reviewed: 05.07.2025
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Thromboangiitis obliterans is an inflammatory thrombosis of small arteries, medium-sized arteries, and some superficial veins, causing arterial ischemia of the distal extremities and superficial thrombophlebitis. The main risk factor is smoking. Symptoms of thromboangiitis obliterans include claudication, non-healing leg ulcers, rest pain, and gangrene. Diagnosis is by clinical examination, noninvasive vascular testing, angiography, and exclusion of other causes. Treatment of thromboangiitis obliterans involves stopping smoking. The prognosis is very good with smoking cessation, but if the patient continues to smoke, the disorder inevitably progresses, often leading to the need for limb amputation.
Thromboangiitis obliterans occurs almost exclusively in smokers and is predominant in men aged 20-40 years.
Only about 5% of cases are registered in women. The disease is more common in people with the HLA-A9 and HLA-B5 genotypes. The prevalence is highest in Asia, the Far and Middle East.
Thromboangiitis obliterans causes segmental inflammation in small and medium-sized arteries and often in the superficial veins of the extremities. In acute thromboangiitis obliterans, occlusive thrombi are accompanied by neutrophilic and lymphocytic infiltration of the inner lining of the vessels. Endothelial cells proliferate, but the internal elastic lamina remains intact. In the intermediate phase, thrombi organize and recanalize incompletely. The middle layer of the vessels is preserved, but may be infiltrated by fibroblasts. In later stages, periarterial fibrosis may develop, sometimes with damage to adjacent veins and nerves.
[ What causes thromboangiitis obliterans?
The cause is unknown, although cigarette smoking is a major risk factor. The mechanism may involve hypersensitivity or toxic vasculitis. Another theory is that thromboangiitis obliterans may be an autoimmune disorder caused by a cell-mediated reaction to human collagen types I and III, which are found in blood vessels.
Symptoms of thromboangiitis obliterans
The symptoms are the same as those of arterial ischemia and superficial thrombophlebitis. Approximately 40% of patients have a history of migratory phlebitis, usually in the superficial veins of the leg or foot. The onset is gradual. The lesions affect the distal vessels of the upper and lower extremities, then progress proximally, culminating in the development of distal gangrene and persistent pain.
A feeling of cold, numbness, tingling, or burning may occur before the development of objective signs of thromboangiitis obliterans disease.
Raynaud's phenomenon is common. There may be intermittent claudication in the affected limb (usually the arch of the foot or leg; less commonly the arm, hand, or thigh), which may progress to pain at rest. If the pain is intense and persistent, the affected leg is usually chronically cold, sweats excessively, and becomes cyanotic, probably due to increased sympathetic tone. Ischemic ulcers develop in most patients and may progress to gangrene.
The pulse is diminished or absent in one or more arteries of the legs and often at the wrist. In young people who smoke and have ulcers of the extremity, a positive Allen test (the hand remains pale after the examiner simultaneously compresses the radial and ulnar arteries and then releases them alternately) confirms the diagnosis. Pallor on elevation and redness on lowering the affected hands, feet, or fingers are often noted. Ischemic ulceration and gangrene, usually of one or more digits, may develop early but not acutely. Noninvasive testing reveals a marked reduction in blood flow and blood pressure in the affected fingers, feet, and toes.
Where does it hurt?
Diagnosis of thromboangiitis obliterans
A presumptive diagnosis is made by collecting anamnesis and physical examination. It is confirmed by the following data:
- the ankle-brachial index (the ratio of systolic blood pressure in the ankle to blood pressure in the arm) or segmental changes in pressure in the upper limbs indicate distal ischemia;
- echocardiography excluded emboli that migrated from the heart cavities;
- blood tests (eg, determination of antinuclear antibodies, rheumatoid factor, complement, anticentromere antibodies, anti-SCL-70 antibodies) exclude vasculitis;
- Antiphospholipid antibody tests rule out antiphospholipid syndrome (although the number of these antibodies may be slightly increased in thromboangiitis obliterans);
- Vasography shows characteristic changes (segmental occlusions of distal arteries in the arms and legs, tortuous collateral vessels around the occlusion, absence of atherosclerosis).
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Who to contact?
Treatment of thromboangiitis obliterans
Treatment involves stopping smoking. Continued smoking inevitably leads to disease progression and severe ischemia, often requiring amputation.
Other measures include avoiding hypothermia, stopping drugs that can cause vasoconstriction, and preventing thermal, chemical, and mechanical injuries, especially from poorly fitting footwear. In patients in the first phase of smoking cessation, iloprost 0.5-3 ng/kg/min intravenously for 6 hours or more may help prevent amputation. Pentoxifylline, calcium channel blockers, and thromboxane inhibitors can be used empirically, but there is no evidence to support their efficacy. Monitoring the disease by measuring antiendothelial antibodies is being studied.
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