Buerger’s disease

A 58-year-old man of eastern European descent with a 12-year history of Buerger’s disease presented with painful acral gangrene of fingers II to V of the left hand and fingers II to IV of the right hand (Panel A). He reported the unprotected use of a commercially available cold pack on both hands after an insignificant blunt trauma as a possible trigger of the lesions. The patient used to be a heavy smoker, but reported to have stopped smoking cigarettes 2 years ago. However, regular consumption of cannabis owing to a chronic pain condition was admitted, and at least sporadic nicotine exposure was suspected clinically. The initial non-invasive vascular work-up demonstrated absent ulnar and weak radial artery pulses bilaterally. The pulse volume recordings of all fingers were severely dampened despite normal tracings at the level of the upper arm and forearm in both extremities. The results indicated distal occlusive disease of the forearm and finger arteries bilaterally, and therefore we decided to refrain from performing additional angiographic imaging due to a lack of interventional therapeutic options. Despite proper local surgical care, intravenous vasodilator therapy with the prostacyclin analogue iloprost, and surgical thoracic sympathectomy, the lesions deteriorated. After a total treatment period of 22 months, healing was finally achieved by adding the phosphodiesterase inhibitor sildenafil to the patient’s therapy in an off-label use as a vasodilator. Thereafter, sildenafil could be tapered off over the course of several weeks without relapse (Panel B).

Panel A Panel B

Buerger’s disease is a non-atherosclerotic segmental inflammatory disease that most commonly affects small and medium-sized arteries and veins. The pathogenesis of this disease condition is still largely unknown, but exposure to tobacco is essential to the initiation and progression of the condition. Clinically, the disease presents with ischemia of the distal blood vessels, leading to claudication or ischemic pain at rest in the affected regions, and finally ischemic ulcerations. ¹ Commonly, treatment is based on strict smoking cessation (including cannabis) as the most effective therapeutic intervention, with surgical therapy as needed. Experience with medical therapies has been described with iloprost and experimental therapeutic angiogenesis. ² Our case demonstrates the successful use of the potent vasodilator sildenafil to treat a patient with Buerger’s disease and advanced gangrene of the fingers. Further study in the setting of controlled clinical trials will be required to determine the role of sildenafil in the treatment of Buerger’s disease.

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