Don’t Forget the Vasculitides: An Approach to Buerger’s Disease
A 50-year-old generally healthy male smoker presents to the ED from hand clinic with atraumatic right index and middle finger pain and color changes for the past 3 days. He initially noticed the symptoms at work this morning. While typing at his desk, he noticed the aforementioned fingers turn blue around their eponychiae. The fingers returned to their normal color within 30 seconds but subsequently became exquisitely painful.
Since then, the same pattern of symptoms has repeated itself numerous times per day with no clear consistent inciting trigger, including temperature changes. The patient states that the symptoms sometimes flare at rest, but that he has them when he uses his hands as well.
On day 1 of his symptoms he went to an urgent care center, where X-rays were done and read as negative for acute pathology. He was referred to the hand clinic and secured a same-day appointment. X-rays were repeated, and again were negative for fracture or dislocation. The clinic then referred the patient to the ED for further investigation.
Physical exam of the hand appeared completely normal. His fingers were warm, well-perfused with brisk capillary refill, and no tenderness to palpation over any joint or phalanx. There were no obvious signs of bony injury or tendon disruption, and the hand had easily palpable radial and ulnar pulses.
The differential at this time included:
A low-flow state secondary to systemic illness
Arterial emboli in the presence of a PFO
Arterial duplexes were obtained of each finger. This study revealed a flattened waveform of the index finger in question, concerning for acute arterial ischemia… in other words, Buerger’s disease!
Vascular surgery was consulted. They recommended heparinization and medical admission for a hypercoagulability workup. Revascularization was considered, but vascular surgery declined this option given the distal location of this patient’s symptoms. The patient was not likely to have enough collateral circulation in the area to make the procedure worthwhile or even possible.
Buerger’s disease (also known as thromboangitis obliterans), defined as non-atherosclerotic small- and medium-vessel vasculitis, typically occurs in male smokers in their 40s (i.e. this patient). Failure to address this disease promptly puts your patient at risk of limb loss due to arterial ischemia, increased pain, permanent motor/sensory deficits, and/or undiagnosed underlying comorbidities.
Typical symptoms include extremity pain and finger cyanosis, particularly with temperature changes. The primary therapy is immediate smoking cessation. Additionally, nicotine replacement therapy is not an option because like smoking, it has the potential to produce vasoconstriction.
Ancillary management options include removal of any constricting clothing, maintenance of systemic perfusion, and analgesia.
Ultimately, this patient may be discharged, but with the knowledge that he/she will require an extensive medical workup to investigate for underlying autoimmune disease, diabetes, embolic sources, and structural/electrophysiologic heart disease. The only exception to this disposition is active arterial ischemia that warrants immediate vascular intervention -- those patients should be admitted.
Either way, prepare your patient with the knowledge that he/she will likely require antiplatelet/anticoagulant therapy.
Do not dismiss history or exam findings you cannot explain as non-life or limb-threatening, even if they’re not present at the time of patient presentation. This patient presented with a normal hand exam and no signs of hypoperfusion, but had a history clearly concerning for hypoperfusion.
The typical Buerger’s disease patient is a male smoker in his 40s with extremity pain, and finger cyanosis, classically triggered by changes in ambient temperature.
This patient needs an extensive workup for underlying causes as well as antiplatelet or anticoagulant therapy. He may be discharged as long as there is no active arterial ischemia that warrants immediate vascular intervention.
Edited by Bassam Zahid, MD