J Korean Rheum Assoc.  2009 Dec;16(4):306-311.

A Case of Systemic Lupus Erythematosus and Abdominal Aorta Thrombosis Associated with Protein C and S Deficiency

• Song JH
• Gweon TG
• Min JK

Abstract

Thrombosis is a well known manifestation in patients with systemic lupus erythematosus, along with lupus anticoagulant, anticardiolipin antibody and anti beta2-glycoprotein I. We describe here a 44-year-old female with an abdominal aorta thrombosis of SLE and the patient had no antiphospholipid antibodies. She had this unusual site of thrombosis and this was associated with protein C and S deficiency. She had no other cause of thrombosis. After anticoagulant treatment, her thrombosis of the abdominal aorta resolved.

Keyword

Systemic lupus erythematosus; Thrombosis; Aorta; Protein C and S deficiency

MeSH Terms

Adult
Antibodies, Anticardiolipin
Antibodies, Antiphospholipid
Aorta
Aorta, Abdominal
beta 2-Glycoprotein I
Female
Humans
Lupus Coagulation Inhibitor
Lupus Erythematosus, Systemic
Protein C
Thrombosis
Antibodies, Anticardiolipin
Antibodies, Antiphospholipid
Lupus Coagulation Inhibitor
Protein C
beta 2-Glycoprotein I

Figure

• 

  Fig. 1. (A) This picture shows peripheral cyanosis of the fingers. (B) This picture shows splinter hemorrhages on the finger nail.

• 

  Fig. 2. Abdominal CT revealed target-appearing concentric wall thickening and mesenteric edema in the proximal jejunum at the time of admission.

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  Fig. 3. (A) Abdominal computed tomography revealed a thrombosis on the abdominal aorta. (B) Abdominal ultrasonography revealed a thrombosis in the abdominal aorta at that time.

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  Fig. 4. There is no thrombosis in the abdominal aorta after anticoagulation treatment.

Reference

References

1. Wahl DG, Bounameaux H, de Moerloose P, Sarasin FP. Prophylactic antithrombotic therapy for patients with systemic lupus erythematosus with or without antiphospholipid antibodies: do the benefits outweigh the risks? A decision analysis. Arch Intern Med. 2000; 160:2042–8.

2. Jobic Y, Provost K, Larlet JM, Mondine P, Gilard M, Boschat J, et al. Intermittent left coronary occlusion caused by native aortic valve thrombosis in a patient with protein S deficiency. J Am Soc Echocardiogr. 1999; 12:1114–6.

3. Onwuanyi A, Sachdeva R, Hamirani K, Islam M, Parris R. Multiple aortic thrombi associated with protein C and S deficiency. Mayo Clin Proc. 2001; 76:319–22.

4. Ju JH, Min JK, Jung CK, Oh SN, Kwok SK, Kang KY, et al. Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE. Nat Rev Rheumatol. 2009; 5:273–81.

5. Cho YP, Lee DH, Jung SM, Jang HJ, Kim JS, Han MS. Vascular Diseases Associated with Protein C and/or S Deficiencies. J Korean Surgical Society. 2002; 62:181–6.

6. Roubey RA. Autoantibodies to phospholipid-binding plasma proteins: a new view of lupus anticoagulants and other "antiphospholipid" autoantibodies. Blood. 1994; 84:2854–67.

7. Esmon NL, Smirnov MD, Safa O, Esmon CT. Lupus anticoagulants, thrombosis and the protein C system. Haematologica. 1999; 84:446–51.

8. Guermazi S, Regnault V, Gorgi Y, Ayed K, Lecompte T, Dellagi K. Further evidence for the presence of anti-protein S autoantibodies in patients with systemic lupus erythematosus. Blood Coagul Fibrinolysis. 2000; 11:491–8.

9. Song KS, Park YS, Kim HK. Prevalence of anti-protein S antibodies in patients with systemic lupus erythematosus. Arthritis Rheum. 2000; 43:557–60.

10. Matsuda J, Gohchi K, Gotoh M, Tsukamoto M, Saitoh N. Plasma concentrations of total/free and functional protein S are not decreased in systemic lupus erythematosus patients with lupus anticoagulant and/or antiphospholipid antibodies. Ann Hematol. 1994; 69:311–5.

11. Silver AS, Shao CY, Ginzler EM. Aortitis and aortic thrombus in systemic lupus erythematosus. Lupus. 2006; 15:541–3.

12. Hughes GR, Khamashta MA. Seronegative antiphospholipid syndrome. Ann Rheum Dis. 2003; 62:1127.

13. Joseph J, Scopelitis E. Seronegative antiphospholipid syndrome associated with plasminogen activator inhibitor. Lupus. 1994; 3:201–3.

14. Lockshin MD, Sammaritano LR, Schwartzman S. Validation of the Sapporo criteria for antiphospholipid syndrome. Arthritis Rheum. 2000; 43:440–3.

15. Erkan D, Lockshin MD. New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol. 2009; 5:160–70.