Thromboangiitis obliterans (Buerger's disease)

Buerger's disease or thromboangiitis obliterans is a primary vasculitis of small and medium caliber blood vessels (arteries and veins). The term "vasculitis" is debated because the involvement is primarily endovascular (thrombus rich in inflammatory cells), with the vascular walls being relatively spared.

It is a rare disease (prevalence of 1 to 10 per 100,000 inhabitants in Europe) primarily affecting men (80%), young (average age: 42 years), smokers (+++ almost constant). Its incidence is increased in India, Korea, Japan, and Israel (Ashkenazi).

Its etiology is unknown, but smoking plays a major triggering and aggravating role.

Clinical Presentation

It generally affects multiple limbs. The revealing signs are a sensation of coldness, numbness, or Raynaud's phenomenon, with pain and trophic disorders appearing later. Superficial venous thromboses (SVT) sometimes precede arterial disorders.

• Rest pain in limbs (80%)
• Ischemic ulcers (75%)
• Sensory disturbances (70%)
• Positive Allen's test: very delayed recoloration (60%)
• Intermittent claudication (60%)
• Raynaud's phenomenon (45%)
• Recurrent and migrating superficial thromboses (40%)

Complementary Examinations

The diagnosis relies solely on clinical presentation and exclusion of differential diagnoses.

Biology

No specificity (no inflammatory syndrome except for superinfections, no autoantibodies [rare descriptions of anticardiolipin antibodies], ...). It is only useful to exclude other pathologies.

Capillaroscopy

It is not specific, but one may find: longer, dystrophic capillaries and marginal hemorrhages.

Arteriography of the Four Limbs

Very suggestive angiographic appearance: distal lesions in the lower limbs (thin arterial trunks, tortuous collaterals), absence of proximal lesions.

It also allows for the elimination of certain differential diagnoses.

Differential Diagnoses to Rule Out

• Embolic pathologies: echocardiogram and arteriography
• Distal arteropathy due to repeated trauma: history (vibrations, hammer disease,…)
• Early atherosclerosis: history (diabetes and hypercholesterolemia), arteriography
• Systemic lupus erythematosus (SLE), scleroderma, polyarteritis nodosa (PAN), hematological diseases, cryoglobulinemia, cold agglutinins, coagulation disorders, ergotism

Therapeutic Management - Treatments

• Complete cessation of smoking (with smoking and psychological follow-up)
  • 95% of patients who quit smoking avoid amputation compared to only 40% of those who do not quit
• Anticoagulation (heparin/AVK) during flare-ups or SVT
• (Antiplatelet agents, corticosteroids, immunosuppressants are sometimes used, without evidence of efficacy)
• Surgery (bypass) is only meaningful when a limb is threatened or a trophic disorder is not healing
• (Effect of VEGF under study)

Bibliography

EMC, traité de médecine AKOS, Elsevier, 2018

Jameson JL et al., Harrison's Principles of Internal Medicine, 20th edition, McGraw Hill Higher Education, 2018