jkra The Journal of the Korean Rheumatism Association J Korean Rheum Assoc 1226-8070 2233-4718 The Korean Rheumatism Association 10.4078/jkra.2009.16.4.306 jkra-16-306 Original Article A Case of Systemic Lupus Erythematosus and Abdominal Aorta Thrombosis Associated with Protein C and S Deficiency SongJun Ho GweonTae Geun MinJun Ki Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea 01 2009 20 01 2009 16 4 306 311 21052009 11082009 17082009 Copyright © 2009 The Korean Rheumatism Association 2009 This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Abstract

Thrombosis is a well known manifestation in patients with systemic lupus erythematosus, along with lupus anticoagulant, anticardiolipin antibody and anti β2-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.

 Systemic lupus erythematosus Thrombosis Aorta Protein C and S deficiency References 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 2000160 20428 Jobic Y Provost K Larlet JM Mondine P Gilard M Boschat J Intermittent left coronary occlusion caused by native aortic valve thrombosis in a patient with protein S deficiency J Am Soc Echocardiogr 199912 11146 Onwuanyi A Sachdeva R Hamirani K Islam M Parris R Multiple aortic thrombi associated with protein C and S deficiency Mayo Clin Proc 200176 31922 Ju JH Min JK Jung CK Oh SN Kwok SK Kang KY Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE Nat Rev Rheumatol 20095 27381 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 200262 1816 Roubey RA Autoantibodies to phospholipid-binding plasma proteins: a new view of lupus anticoagulants and other "antiphospholipid" autoantibodies Blood 199484 285467 Esmon NL Smirnov MD Safa O Esmon CT Lupus anticoagulants, thrombosis and the protein C system Haematologica 199984 44651 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 200011 4918 Song KS Park YS Kim HK Prevalence of anti-protein S antibodies in patients with systemic lupus erythematosus Arthritis Rheum 200043 55760 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 199469 3115 Silver AS Shao CY Ginzler EM Aortitis and aortic thrombus in systemic lupus erythematosus Lupus 200615 5413 Hughes GR Khamashta MA Seronegative antiphospholipid syndrome Ann Rheum Dis 200362 1127 Joseph J Scopelitis E Seronegative antiphospholipid syndrome associated with plasminogen activator inhibitor Lupus 19943 2013 Lockshin MD Sammaritano LR Schwartzman S Validation of the Sapporo criteria for antiphospholipid syndrome Arthritis Rheum 200043 4403 Erkan D Lockshin MD New approaches for managing antiphospholipid syndrome Nat Clin Pract Rheumatol 20095 16070 Figures

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

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

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

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