From the Guidelines
Buerger's disease diagnosis is primarily based on clinical presentation, angiographic findings, and the exclusion of other potential causes, with a strong emphasis on the patient's history of tobacco use, as stated in the ACR Appropriateness Criteria for nonatherosclerotic peripheral arterial disease 1.
Key Diagnostic Elements
- The disease typically affects young smokers, usually under age 45, with a strong male predominance.
- Clinical diagnosis requires evidence of distal extremity ischemia such as claudication, rest pain, ulcers, or gangrene, particularly in the hands and feet.
- Angiographic findings are distinctive, showing segmental occlusions of small and medium-sized arteries with "corkscrew" collaterals and sparing of the proximal vessels, as described in the context of nonatherosclerotic peripheral arterial disease 1.
Supporting Criteria
- Migratory superficial thrombophlebitis
- Raynaud's phenomenon
- Upper limb involvement
Exclusion Criteria
- Other causes of vasculitis
- Hypercoagulable states
- Diabetes
- Proximal sources of emboli
- Autoimmune diseases
Diagnostic Considerations
Biopsy findings, though not always necessary, typically show acute and chronic thrombus with minimal inflammation in the vessel wall and preserved internal elastic lamina. The strongest diagnostic factor is the relationship to tobacco use, as symptoms almost invariably occur in active smokers and improve with complete tobacco cessation, which remains the cornerstone of treatment, as implied by the association of Buerger disease with heavy tobacco smoking in the ACR Appropriateness Criteria 1.
From the Research
Diagnostic Criteria for Buerger's Disease
The diagnostic criteria for Buerger's disease have been established based on various studies, including those by 2, 3, 4, 5, and 6. The key criteria include:
- History of smoking
- Onset before the age of 50 years
- Infrapopliteal arterial occlusive disease
- Either upper limb involvement or phlebitis migrans
- Absence of atherosclerotic risk factors other than smoking
Detailed Diagnostic Criteria
A more detailed set of criteria has been proposed by 4, which includes:
- A major criterion: history of active tobacco smoking
- Minor criteria:
- Disease onset at age less than 45 years
- Ischemic involvement of the lower limbs
- Ischemic involvement of one or both of the upper limbs
- Thrombophlebitis migrans
- Red-blue shade of purple discoloration on edematous toes or fingers
- A suspected diagnosis of Buerger's disease is confirmed in the presence of a major criterion plus four or more minor criteria
Clinical Features and Diagnosis
The clinical features of Buerger's disease include distal extremity ischemia, ischemic ulcers, or frank gangrene of the toes or fingers, as reported by 2, 3, and 6. The diagnosis is based on a combination of clinical evaluation, angiographic findings, and histopathological features. The angiographic findings in Buerger's disease are characterized by "corkscrew," "spider legs," or "tree roots" patterns, but are not pathognomonic, as noted by 5.
Importance of Tobacco Abstinence
Tobacco abstinence is crucial in the management of Buerger's disease, as it can result in disease quiescence, as reported by 2, 3, and 6. The cessation of smoking is the only means of stopping the disease progression, and patients who continue to smoke are at risk of major amputation, as noted by 3.