Buerger disease Flashcards
A 30-year-old Asian man comes to the clinic due to painful, ulcerated fingers. His symptoms began with pain and discoloration of the fingers several weeks ago, which progressed to ulceration at the fingertips. He also reports pain in the hands that occurs with activity and is relieved by rest. Medical history is unremarkable. The patient has smoked 2 packs of cigarettes a day for the last 5 years. He occasionally drinks alcohol but does not use illicit drugs. Vital signs are within normal limits. No heart murmurs are heard and the lungs are clear. Examination of the hands is shown in the exhibit. Radial pulses are diminished. There is erythema and tenderness along a superficial vein of the left forearm. Hemoglobin A1c and a fasting lipid panel are within normal limits. What is the most likely pathology responsible for this patinet’s findings?
This patient’s presentation is consistent with thromboangiitis obliterans (Buerger disease), a segmental, inflammatory vasculitis that affects the small and medium sized arteries and veins of the distal extremities. It typically affects young, heavy smokers (age <45) and is more common in men. Toe and/or finger ischemia and ischemic ulcerations are the most common manifestation, and patients with involvement of proximal arteries can develop claudication affecting the lower legs, feet, or hands. Raynaud phenomenon and superficial thrombophlebitis (erythema, tenderness along veins) may also occur as early manifestations of the disease. In thromboangiitis obliterans, chronic exposure to tobacco products is thought to cause direct endothelial injury or trigger a delayed-type hypersensitivity reaction against the endothelium. Histopathology shows highly cellular, inflammatory intraluminal thrombi (ie, containing neutrophils, multinucleated giant cells) in the arteries and veins with sparing of the vessel wall and internal elastic lamina. This segmental, thrombosing vasculitis often extends contiguously into veins and nerves, a feature rarely seen in other types of vasculitis.
What is Buerger’s disease (Thromboangiitis Obliterans)?
A segmental, inflammatory, non-atherosclerotic vasculitis affecting small- and medium-sized arteries and veins, strongly linked to heavy smoking.
What is the most critical risk factor for Buerger’s disease?
Tobacco use; smoking cessation is the only definitive treatment to halt disease progression.
What is the hallmark histological feature of Buerger’s disease?
Segmental vasculitis with inflammatory thrombi, sparing the vessel wall and internal elastic lamina.
What is the gold standard diagnostic test for Buerger’s disease?
Angiography, which shows segmental occlusions and corkscrew collaterals.
What are the main clinical manifestations of Buerger’s disease?
Digital ischemia, ischemic ulcers, Raynaud phenomenon, superficial migratory thrombophlebitis, and claudication.
What are the key diagnostic criteria for Buerger’s disease?
Age <45 years, heavy tobacco use, distal extremity ischemia, non-atherosclerotic arterial occlusions, and exclusion of other autoimmune or embolic diseases.
What is the most effective treatment for Buerger’s disease?
Absolute smoking cessation, which is the only intervention that prevents disease progression.
What medications can help manage symptoms in Buerger’s disease?
Iloprost (prostacyclin analog) improves pain and ulcer healing; calcium channel blockers for Raynaud phenomenon.
Which vascular structures are affected in Buerger’s disease?
Small- and medium-sized arteries and veins, often with inflammation extending into adjacent nerves.
What is the key distinguishing feature of Buerger’s disease compared to atherosclerosis?
Buerger’s disease occurs in younger patients without typical atherosclerotic risk factors and lacks lipid-laden plaques.
What are some conditions that should be ruled out when diagnosing Buerger’s disease?
Atherosclerosis, giant cell arteritis, Raynaud disease, scleroderma, polyarteritis nodosa, and thromboembolic disease.
What findings on angiography suggest Buerger’s disease?
Corkscrew collaterals and segmental occlusions of small- and medium-sized arteries.
What surgical intervention may be needed in severe Buerger’s disease?
Amputation in cases of extensive tissue necrosis or gangrene.
What is a unique feature of the inflammatory process in Buerger’s disease?
The inflammation extends into contiguous veins and nerves, unlike most other vasculitides.
