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

K Lhotta

Division of Clinical Nephrology, Department of Internal Medicine, Innsbruck University Hospital, Innsbruck, Austria, karl.lhotta{at}uibk.ac.at

R Würzner

Institute of Hygiene and Social Medicine, University of Innsbruck, Innsbruck, Austria

A R Rosenkranz

Division of Clinical Nephrology, Department of Internal Medicine, Innsbruck University Hospital, Innsbruck, Austria

R Beer

Department of Neurology, Innsbruck University Hospital, Innsbruck, Austria

A Rudisch

Department of Diagnostic Radiology I, Innsbruck University Hospital, Innsbruck, Austria

F Neumair

Department of Internal Medicine, Brixen Hospital, Brixen, Italy

G Mayer

Division of Clinical Nephrology, Department of Internal Medicine, Innsbruck University Hospital, Innsbruck, Austria

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.

Key Words: complement C4 • immunoadsorption • lupus erythematosus • mycophenolate mofetil • vasculitis

Lupus, Vol. 13, No. 2, 139-141 (2004)
DOI: 10.1191/0961203304lu489cr
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This article has been cited by other articles:


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Y. Yang, K. Lhotta, E. K. Chung, P. Eder, F. Neumair, and C. Y. Yu
Complete Complement Components C4A and C4B Deficiencies in Human Kidney Diseases and Systemic Lupus Erythematosus
J. Immunol., August 15, 2004; 173(4): 2803 - 2814.
[Abstract] [Full Text] [PDF]


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