History and exam

Key diagnostic factors

common

presence of risk factors

Key risk factors include age <40 years, male sex, and history of smoking.

Other diagnostic factors

common

paraesthesias/cold sensation/cyanosis in limb or finger

Paraesthesias/cold sensation/cyanosis occur in approximately 37% of patients.[24]

ulceration/gangrene

Ulceration or gangrene on the distal phalanges may be noted. [Figure caption and citation for the preceding image starts]: Fingertip ulceration in a woman who smokesFrom the collection of Matthew J. Metcalfe and Alun H. Davies [Citation ends].com.bmj.content.model.Caption@1fc32cb5

Occurs in approximately 19% of patients.[24]

claudication

Plantar claudication occurs in approximately 15% of patients, and sural claudication occurs in approximately 16% of patients.[24]

Claudication of the arch of the foot may be described by the patient.

rest pain

Occurs in approximately 10% of patients.[24]

The lower limb is often painful and can be eased by hanging the leg over the edge of the bed at night. This suggests ischaemia and is not specific for Buerger's disease.

superficial thrombophlebitis

A history of recurrent superficial thrombophlebitis of either the arms or legs may be given.

cold limb or finger

A cold ischaemic limb or finger is present in acute ischaemia. [Figure caption and citation for the preceding image starts]: A cold, ischaemic middle finger in a woman who smokesFrom the collection of Matthew J. Metcalfe and Alun H. Davies [Citation ends].com.bmj.content.model.Caption@7e418e94

pale limb or finger

A pale ischaemic limb or finger is present in acute ischaemia.

absence of distal pulses

Popliteal pulses are present, but foot pulses are absent.

In the forearm, brachial and distal forearm pulses may be absent.

positive Allen test

Allen test may detect Buerger's disease in 63% of patients.[5] Performed by occluding both radial and ulnar arteries and observing whether the patient's hand becomes ischaemic. The pressure on the radial and ulnar arteries is then released 1 artery at a time. The release of each artery should reperfuse the hand individually. A negative Allen test reveals no ulnar or radial artery occlusion.

An abnormal test in a young patient is highly suggestive of Buerger's disease, although may be negative in 25% of patients; a positive test in a patient with lower limb disease may indicate the presence of upper limb disease.

uncommon

joint arthritis

12.5% of patients may give a history of recurrent episodes of large joint arthritis before their arterial occlusion presentation.[25]

duration of joint symptoms up to 2 weeks

Single-joint inflammation, most often described affecting the wrists and knees, lasting up to 2 weeks. The diagnosis of Buerger's disease is often not made until 10 years after the joint symptoms.

Risk factors

strong

smoking

An association with tobacco smoking has suggested a possible hypersensitivity to tobacco constituents. Less than 5% of Buerger's disease patients are non-smokers. Smoking cessation reduces amputation risk.[5] A return to smoking following cessation may lead to a flare-up of the disease. Risk of Buerger's disease is thought to be increased by 'bidi' (a home-made cigarette consisting of low-quality tobacco and smoked without filters) smoking, which is possibly related to cannabis arteritis associated with cannabis consumption.[9][10]

Smoking only 1 or 2 cigarettes a day, using smokeless tobacco (chewing tobacco), or using nicotine replacement therapy may all keep the disease active.[19][20]

age <40 years

Buerger's disease is most common in those aged <40 years. In one study, the median age of onset was 36 years.[2] 

region of origin: southeast Mediterranean, Middle East, and Far East

Buerger's disease is most commonly seen in people of southeast Mediterranean origin and of Middle and Far Eastern origin.[1] It is becoming less common in Western countries.

male sex

Although Buerger's disease is more commonly seen in men, approximately 23% of patients diagnosed with Buerger’s disease are women.[2][5][6]

periodontal infection

There is a prevalence of anti-cardiolipin antibodies in patients with Buerger’s disease with high levels associated with increased morbidity.[21] Anti-cardiolipin antibodies have an association with periodontal infection.[22]

weak

human leukocyte antigen (HLA) haplotypes

In areas of India and Japan where Buerger's disease has a high prevalence, an association with HLA-DRB1*1501 has been identified.[23] However, no HLA haplotype has been associated with Buerger's disease in North America.

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