Etiology and Pathophysiology:
Smoking is very closely related to Buerger's disease and
smoking history is one of the criterion for diagnosing the disease. In
general if the patient absolutely abandons smoking the course of the disease
will be invariably benign, but if smoking continues any treatment will
ultimately be futile. Though "passive smoking" has adverse effect
on cardiovascular system, non smokers should never develop the disease.
Active smokers can be indentified by measuring levels of continine, the
major metabolite of nicotine in urine. Since all smokers do not develop
the disease an immunopathogenesis is considered probable. It has been
proposed in Japanese that presence of a gene linked to some HLA antigens
might control the susceptibility to the disease.
Socioeconomic conditions, work environment may also play
in etiology as the disease is seen more in out door and manual workers.
Hypercoagulable state has been observed in association with the disease.
Hepatitis B Virus and rickettssiosis may contribute to pathogenesis, but
this role is uncertain.
Buergers disease is an inflammatry occlusive disease which
involves all layers of medium sized and small arteries of the extremitiles.
Involved superficial veins bear a close resemblance to those in the affected
artery. Majority of the patients develop critical limb ischemia with trophic
lesions are distal to ankle, the anklebrachial doppler index could be
normal in early stage. Toe pressures can be measured and if it is less
than 30 mm Hg, the healing of ulcers is unlikely. The disease though commences
peripherally, may gradually extend proximately occluding the larger arteries.
Clinical Presentation :
varies with the stage of the disease. The patients may present with foot
claudication and later with calf claudication. Gangrene and ulceration
may follow the above symptoms, but many times they may occur without previous
history of claudication. The stepwise progession of the disease as seen
in atherosclerasis may not occur in Buergers disease. Parasthesia, coldness
and skin colour changes are common complaints. Dependent rubor and slow
venous filling are commonly seen. Gangrene and ulceration usually follow
minor trauma and with development of secondary infection they may progress
proximally and are associated with intolerable rest pain.
Recurrent superficial thrombophlebitis marked by redness
and tenderness over the affected vain, can occur in arm, leg or the foot.
The symptoms usually disappear over 2-3 weeks, leaving behind blackish-brown
pigmentation. "Phlebitis migrans" is characteristic of Buergers
disease, but is often missed both by the patient and the doctor.
Of 255 patients treated by shionaya from an institution
in India 98% were males. The major presenting symptoms were.
- Parasthesia, Coldness, Cyanosis - 37%
- Gangrene or Ulcer - 18%
- Foot Claudication - 15%
- Calf Claudication - 16%
- Rest pain - 10%
- Thrombophlebitis - 3%
On continuous follow up-72% develop Ulcer/Gangrene, 42% develop phlebitis
migrans and about 90% eventually have upper extremity involvement. In
the above series 83% had 3 or 4 limb involvement and 17% had 2 limb involvement
and NONE had a single limb involvement.
In our experience with about 80 patients with Buergers disease, we have
not seen a single patient without ulcer or gangrene!
The criteria for diagnosis of Burgers disease include (1) History of
smoking (2) Onset before the age of 50 years (3) Infrapopliteal arterial
occlusive disease (4) Either upper limb involvement or phlebitis migrans
(5) Absence of atherosclerotic risk factors other than smoking. Arteriographic
findings serve as supporting evidence and will be discussed later.
Though history and physical exam are sufficient to diagnose Buerger's
disease, diagnostic studies are required for objective evalution and to
select appropriate therapy.
Duplex Scanning : is useful in initial
evaluation of the patient. Since these patient may have multiple "skip"
lesions and also have significant distal disease, arteriogram is a must
before any interventional therapy is planned.
ARTERIOGRAM remains the "gold standard" for evaluation of arterial
occlusive disease. Digital subtraction angiography is better in evaluation
of distal occlusions. As mentioned above multisegmental occlusion of distal
limb arteries are characteratic of Buergers disease. There is usually
an extensive network of collaterals and these have a "corkscrew"
or "root like oppearance".
About 60% of patents have occulusion of infrapopliteal arteries, about
32% have involvement of femoral popliteal segment and about 8% have aortoiliac
disease. Arterial thromboses can extend proximally in aortic lesions causing
infrarenal occlusion of the aorta. Involvement of other arteries like
visceral vessels, coronary arteries are extremely rare and hence these
patients have a normal life span.
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