A challenging case of thromboangiitis obliterans

Thromboangiitis obliterans, or Buerger disease, is a rare, idiopathic, occlusive vascular disease that is strongly associated with smoking and tobacco use. Among patients with peripheral vascular disease, reported prevalence ranges from 45% to 63% in India and 16% to 66% in Korea and Japan, to 0.5% to 5.6% in Western Europe and 0.75% in North America. Establishing the diagnosis and managing the disease in a timely way pose significant challenges because an extensive workup is required to rule out other causes of arterial occlusion, and no clear evidence-based management guidelines are available. The case of a young female smoker who was diagnosed with thromboangiitis obliterans after a thorough workup for distal arterial occlusions of the lower limbs highlights some of the diagnostic, logistic, and therapeutic challenges encountered with this uncommon disease. A challenging case of thromboangiitis obliterans Contributions from several hospital services, including vascular surgery, acute pain management, and rheumatology, were needed to resolve the diagnostic and therapeutic complexities of an uncommon occlusive vascular condition in a young female smoker. Dr Abdullah is in PGY-1 of the internal medicine residency program at the University of British Columbia. Dr Hayden is in PGY-3 of the internal medicine residency program at UBC. Dr Gabana is a clinical assistant professor in general internal medicine at UBC and a consultant general internist at Royal Columbian Hospital in New Westminster, BC. Dr Appleton is a clinical instructor in internal medicine at UBC and a consultant rheumatologist at Royal Columbian Hospital. This article has been peer reviewed. Case data A previously healthy 23-year-old female was being treated as an outpatient for seronegative asymmetric oligoarthritis. She had received trials of methotrexate, hydroxychloroquine, and sulfasalazine to suboptimal effect, and had experienced an initially positive response to prednisone. Her symptoms began worsening a few months later and she presented with the new complaint of a cold sensation in her extremities. A vascular ischemic process was suspected and urgent referral to vascular surgery was made. In the interim, she was diagnosed with Raynaud phenomenon at a visit to the emergency department. At her follow-up clinic visit, she was found to have worsening claudication, cold and mildly cyanotic feet, and a nongangrenous 1-cm dark purple skin lesion on the lateral sole of her foot. Because of concerns about progressive and worsening arterial ischemia, she was admitted to hospital to expedite the investigations needed for suspected vasculitis and consideration of other vasculopathies. Aside from asthma and intermittent diarrhea, her medical history was unremarkable. Notably, the patient was a heavy smoker and had increased her tobacco consumption leading up to presentation. Following admission to hospital, the lesion on the patient’s foot expanded to become a 5-cm black lesion with necrotic appearance and was accompanied by evidence of digital ischemia ( Figure 1 ). The patient later developed multiple lesions on the soles bilaterally, which were consistent with superficial phlebitis. Biopsy of the primary lesion revealed mild perivascular lymphocytic and inflammatory cell infiltrate with no evidence of a classic vasculitis or findings suggestive of any alternate diagnosis. Based on the initial presentation and investigations, the working diagnosis was primary vasculitis with seronegative arthropathy. The differential diagnosis had originally included secondary vasculitis, atherosclerotic peripheral arterial disease, thrombosis due to a hypercoagulable state, and proximal source of emboli. Basic hematologic and metabolic parameters, including lipid levels and renal and liver function, were within the normal range. Aside from an ele-