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BUERGER' S DISEASE

(THROMBOANGIITIS OBLITERANS)
Inflammatory Diseases
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Buergers disease is a segmental, inflammatory, occlusive, vascular disease that


usually affects the medium and small sized vessels of the upper and lower extremities
1&2
. The lower extremities are involved in most cases, and the upper extremities in 4050% of cases 3. The occlusions are made by inflammatory thrombi, which differ from
normal thrombi in that they include many inflammatory cells, with neutrophil
polymorphs being the most prevalent 2. Male, heavy tobacco users, 25-50 years old
are mostly affected1. Buergers disease is non-atherosclerotic, but in practice the two
conditions are often confused. Similarities and differences are highlighted later in the
text. The aetiology of the disease is unknown.
PATHOLOGY
There are three phases in this thrombocytic disease. The acute phase is characterised
by inflammation. Firstly, platelet adhesiveness is increased and the lumen is
obstructed by a highly cellular thrombus, which contains a dense mass of neutrophil,
lymphocytes and giant cells. The affected areas are interspersed along the blood
vessel. Mononuclear leukocytes and lymphocytes remain in the lumen, forming
foamy giant cells 1&4. At the periphery of the thrombus, microabscesses that contain
polymorphs and multinucleated giant cells may form 1. The inflammatory cells then
migrate into the surrounding vessel wall, involving the intima, media and adventitia.
The inflammatory reaction spreads to the vessel coats, adjacent vessels and nerves. In
the subacute phase, inflammatory cells are less involved. Perivascular fibrosis,
recanalization of the thrombus and vascularization of the blood vessel wall is
characteristic 1&5. In the late phase the thrombus is often organised and recanalized.
The fibrosis spreads and may result in the binding together of arteries, veins and
nerves by the fibrous cords 2&4. Overall, in Buergers disease the vessel wall remains
intact. In atherosclerosis and other forms of vasculitis, the internal elastic lamina and
media of the blood vessel are usually affected 1&4.
CONTRIBUTORY AETIOLOGICAL FACTORS
SMOKING
The cause of Buergers disease is unknown. However, the use of or exposure to
tobacco is central to disease initiation and progression. 95% of Buergers patients are
smokers, and exacerbations of the disease usually follow exposure to tobacco 1&3. In

some cases, nicotine replacement therapy has been sufficient in maintaining disease
activity 4. An overall increase in smoking among women may be responsible for their
increased prevalence of Buergers disease 1. Buergers is largely self-limiting as
sustained smoking cessation, shows Buergers patients to have a survival time which
is comparable to that of the normal population 1&2. However, if smoking continues,
gangrene, distal ischemia and amputation may result 4&5 (See Fig 1&2 &3).
It is thought that the link between smoking and Buergers may be due to patients
being hypersensitive to tobacco products 3. Tobacco glycoprotein has been implicated
as a cause of increased vascular reactivity 2.

Fig1 & 2: Shows gangrene in a Buerger's patients foot, following thrombosis.


Photographs courtesy of Dr. Luqmani, Western General Hospital, Edinburgh,
Scotland.

Fig 3: The result of amputation


INFECTIVE AGENTS
It is thought that infection may provoke the intense inflammatory reaction found in
the thrombus. Fungi and Rickettsia have been implicated as they have been recovered
from lesions 1.
IMMUNOLOGICAL MECHANISMS
Cell mediated hypersensitivity to collagen types 1 and 3 have been found in
Buergers patients 1&4. Anticollagen and antiendothelial antibodies have also been
identified 5&6. The level of antiendothelial antibodies provides information on the
extent of disease activity 7.
GENETICS
A possible genetic predisposition has also been proposed, as Buergers patients show
and increase in the HLA-A9 and HLA-B5 antigen 1&3&4. In addition, there is greater
prevalence in certain geographical areas, such as the Orient, India, South Asia and
various Eastern European countries. Buergers disease also shows a familial
occurrence 1.
DIFFERENTIAL DIAGNOSIS
In addition to the comments above the following should be taken into consideration
when differentiating between Buergers disease, atherosclerosis and other forms of
vasculitis. The distal nature of Buergers disease is of diagnostic importance as it
usually affects the medium and peripheral vessels and progresses proximally 7. On the
other hand, atherosclerosis is usually found in the visceral or cerebral blood vessels 1.
Those affected by Buergers disease are also usually younger that those presenting
with symptoms associated with atherosclerosis 1&3.
References