Extensive medium-vessel vasculitis with SLE: an unusual association

Abstract

Systemic lupus erythematosus (SLE) is a multisystem disease of autoimmune origin. Vasculitis is often seen with SLE, but is usually limited to small vessels alone. The primary pathology in SLE-related vasculitis is leukocytoclastic vasculitis. Medium- and large-vessel vasculitis in association with SLE is distinctly uncommon and is limited to occasional case reports only. In addition, reports of medium-vessel vasculitis with SLE generally describe involvement of a single vessel bed. We report a rare case of extensive vasculitis involving medium-sized vessels in a patient with SLE. Our patient presented with classic signs and symptoms of SLE and satisfied a majority of the American College of Rheumatology criteria for SLE. She also had toe gangrene at presentation and developed paraplegia during the hospital stay. Radiologic studies showed evidence of diffuse medium-sized vessel vasculitis. The patient was treated with monthly pulse cyclophosphamide and high-dose prednisolone. Follow-up showed no new features of vasculitis and improvement in the signs and symptoms of SLE. Her paraplegia showed no response to treatment. This case stresses that medium-sized vessel occlusion anywhere in the body can occasionally occur due to vasculitis in a patient with SLE.