Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus

Abstract

We describe the case of a female patient with hereditary complete C4 deficiency and systemic lupus erythematosus. She had suffered from lupus nephritis in early childhood. At the age of 23 years she developed severe lupus with skin disease and life-threateningcerebral vasculitis. Her cerebral disease was unresponsiveto high-dosesteroids, intravenousimmunoglobulin, fresh frozen plasma and plasma exchange. Improvement was achieved with immunoadsorption in combination with mycophenolate mofetil. The patient made a complete recovery and is maintained in complete remission on mycophenolate and low-dose steroids.

Keywords

complement C4 immunoadsorption lupus erythematosus mycophenolate mofetil vasculitis

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