Cerebral vasculitis in a patient with hereditary complete C4 deficiency and systemic lupus erythematosus-Reference-Cited by-同舟云学术

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

Published:2004-02 Issue:2 Volume:13 Page:139-141
ISSN:0961-2033
Container-title:Lupus
language:en
Short-container-title:Lupus

Author:

Lhotta K¹,Würzner R²,Rosenkranz A R³,Beer R⁴,Rudisch A⁵,Neumair F⁶,Mayer G³

Affiliation:

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

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

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

4. Department of Neurology, Innsbruck University Hospital, Innsbruck, Austria

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

6. Department of Internal Medicine, Brixen Hospital, Brixen, Italy

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.

Publisher

SAGE Publications

Subject

Rheumatology

Link

http://journals.sagepub.com/doi/pdf/10.1191/0961203304lu489cr

Reference16 articles.

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2. Complete inherited deficiency of the fourth complement component in a child with systemic lupus erythematosus and his disease-free brother in a North African family

3. Characterization of non-expressed C4 genes in a case of complete C4 deficiency: identification of a novel point mutation leading to a premature stop codon

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