Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus.
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-threatening cerebral vasculitis. Her cerebral disease was unresponsive to high-dose steroids, intravenous immunoglobulin, 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.
Division of Clinical Nephrology, Department of Internal Medicine, Innsbruck University Hospital, Innsbruck, Austria. karl.lhotta@uibk.ac.at
K
K
Lhotta
R
R
Würzner
A R
AR
Rosenkranz
R
R
Beer
A
A
Rudisch
F
F
Neumair
G
G
Mayer
D000328:Adult; D002561:Cerebrovascular Disorders / Q000209:etiology* / Q000628:therapy; D003181:Complement C4 / Q000172:deficiency*; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008181:Lupus Nephritis / Q000150:complications; D009173:Mycophenolic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D020293:Vasculitis, Central Nervous System / Q000209:etiology* / Q000628:therapy
D003181:Complement C4; D011239:Prednisolone; D009173:Mycophenolic Acid