Background: Vasculopathy in systemic lupus erythematosus is a rare form of vascular involvement characterized by non-inflammatory vascular injury with the accumulation of immune complexes in the walls of the arteries, resulting in luminal narrowing. While previous reports have demonstrated vasculitis in the large vessels or vasculopathy in the small vessels, vasculopathy in large vessels has not yet been reported.
Case presentation: We present the case of a 43-year-old Japanese woman with peripheral large vessel vasculopathy associated with systemic lupus erythematosus. She presented a 7-year history of progressive headaches and intermittent claudication, although she had no atherosclerotic risk factors. Vascular ultrasonography and enhanced computed tomography showed multiple vascular stenoses and occlusion. The histological findings of her left temporal artery revealed narrowing of the lumen caused by intimal thickening without inflammatory cells and the deposition of immunoglobulin G, complement component 3, and fibrinogen in the wall of the intima. Beraprost and cilostazol improved arterial occlusion without immunosuppressive therapy.
Conclusions: Large vessel vasculopathy should be considered another potential cause of arterial stenoses and occlusion in patients with lupus when they have peripheral arterial disease despite having no atherosclerotic risk factors.
Keywords: Large vessel vasculopathy; Systemic lupus erythematosus; Vasculitis; Vasculopathy.