Systemic lupus erythematosus – a report of an unusual case with hemorrhagic manifestations
Authors:
D. Jílek 1,3; Z. Burianová 1; J. Kourek 1; A. Vašková 2; J. Laštůvka 1
Authors‘ workplace:
Interní oddělení Masarykovy nemocnice o. z., Krajská zdravotní a. s., Ústí nad Labem
1; Oddělení klinické hematologie Masarykovy nemocnice o. z., Krajská zdravotní a. s., Ústí nad Labem
2; Centrum imunologie a mikrobiologie Zdravotního ústavu se sídlem v Ústí nad Labem
3
Published in:
Čes. Revmatol., 23, 2015, No. 1, p. 34-38.
Category:
Case Report
Overview
The present case report describes a patient with SLE manifesting as a severe disease with a variety of organ complications and the development of life-threatening coagulopathy based on the presence of autoantibodies against coagulation factor VIII and von Willebrand factor. The condition was controlled with difficulties using combined immunosuppression, iv administration of immunoglobulins and anti-CD20 monoclonal antibodies (rituximab), along with long-term administration of recombinant activated coagulation factor VIIa (NovoSeven®). The disease activity was reduced during the treatment and the patient is now treated with maintenance therapy with glucocorticoids.
Key words:
SLE, hemorrhagic event, antibodies against Factor VIII and von Willebrand factor, rituximab
Sources
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Dermatology & STDs Paediatric rheumatology RheumatologyArticle was published in
Czech Rheumatology
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