Buerger's Disease

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.

  1. Parasthesia, Coldness, Cyanosis - 37%
  2. Gangrene or Ulcer - 18%
  3. Foot Claudication - 15%
  4. Calf Claudication - 16%
  5. Rest pain - 10%
  6. 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.

Diagnostic Studies:

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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