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

1. Horák P, Tegzová D, Závada J et al. Doporučení České revmatologické společnosti pro diagnostiku a sledování nemocných se systémovým lupus erythematodes. Čes Revmatol 2013; 21(2): 59–70.

2. Sherer Y, Shoenfeld Y. Autoantibodies guide in systemic lupus erythematosus. BioRad Laboratories 2007.

3. D´Cruz DP, Khamashta MA, Hughes GRV. Systemic lupus erythematosus. Lancet 2007; 369: 587–596.

4. Ishikawa T, Tsukamoto N, Suto M, Uchiumi H, Mitsuhashi H, Yokohama A, Maesawa A, Nojima Y, Naruse T. Acquired hemophilia A in a patient with systemic lupus erythematosus. Intern Med 2001; 40(6): 541–543.

5. Bidwell E, Denson KWE, Dike GWR, Augustin R, Lloyd GM. Antibody nature of the inhibitor to antihemophilic globulin (Factor VIII). Nature 1966; 210: 746–747.

6. Ahmed AEE. Autoantibodies to coagulation factors and bleeding disorders. Clin Rev Allergy Immunol 1998; 16: 313–319.

7. Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. Thromb Haemost 1981; 45: 200–203.

8. Lollar P. Pathogenic antibodies to coagulation factors. Part one: factor VIII and factor IX. J Thromb Haemost. 2004; 2(7): 1082–95.

9. Lollar P. Pathogenic antibodies to coagulation factors. Part II. Fibrinogen, prothrombin, thrombin, factor V, factor XI, factor XII, factor XIII, the protein C system and von Willebrand factor. J Thromb Haemost 2005; 3(7): 1385–91.

10. Akahoshi M, Aizawa K, Nagano S, Inoue H, Sadanaga A, Arinobu Y, Niiro H, Nakashima H. Acquired hemophilia in a patient with systemic lupus erythematosus: a case report and literature review. Mod Rheumatol 2008; 18(5): 511–515.

11. Hong S, Lee J, Chi H, Lee C, Nah S, Kim Y, Oh J, Moon H, Yoo B. Systemic lupus erythematosus complicated by acquired von Willebrand's syndrome. Lupus 2008; 17(9): 846–848.

12. Jiménez OE, Fragoso LS, Gabayet R.M. Aquired inhibitors of coagulation in a patient with systemic lupus erythematosus and antiphospholipid antibodies: response to rituximab. Reumatol Clin 2008; 4(2): 74–76.

13. Cugno M, Gualtierotti R, Tedeschi A, Meroni PL. Autoantibodies to coagulation factors: From pathophysiology to diagnosis and therapy. Autoimmunity Rev 2014; 13: 40–48.

Labels
Dermatology & STDs Paediatric rheumatology Rheumatology
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