Uncommon arteriopathies: what the vascular surgeon needs to know

Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as Marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu's arteritis, extracranial giant cell arteritis, and Behçet's disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. Patients with uncommon arterial disorders may present to the vascular surgeon with common surgical problems, including intermittent claudication, renovascular hypertension, Raynaud's phenomenon, and aneurysmal disease. However, the disease manifestations, expected course, and outcomes may be much different than more common arterial disorders and this can have important surgical implications. This review centers around several interesting cases and the differential diagnoses that should be considered when encountering an unusual clinical presentation. Reference is made to the literature for diagnostic criteria, clinical pearls, and how to avoid pitfalls in the evaluation and management of patients with unusual arteriopathies.