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Lee, Sim, Noh, Park, Tae, Lim, Jun, Ryu, Chun, Lee, Chang, and Moon: A Case of Primary Antiphospholipid Syndrome with Pulmonary Thromboembolism
Case Report
Tuberculosis and Respiratory Diseases

Tuberculosis and Respiratory Diseases 2007; 63(1): 72-77.

Published online: 31 July 2007

DOI: https://doi.org/10.4046/trd.2007.63.1.72

A Case of Primary Antiphospholipid Syndrome with Pulmonary Thromboembolism

Jae Beom Lee, M.D., Yun Su Sim, M.D., Young Wook Noh, M.D., Hye Sung Park, M.D., Chung Hyun Tae, M.D., So Yeon Lim, M.D., Yoon Hee Jun, M.D., Yon Ju Ryu, M.D., Eun Mi Chun, M.D., Jin Hwa Lee, M.D., Jung Hyun Chang, M.D., Jin Wook Moon, M.D.

Department of Internal Medicine, College of Medicine, Ewha Womans University, Seoul, Korea.

Address for correspondence: Jin Wook Moon, M.D. Department of Internal Medicine, College of Medicine, Ewha Womans University 911-1 Mok-6-dong, Yangcheon-gu, Seoul, Korea. Phone: 82-2-2650-2507, Fax: 82-2-2655-2076, greenzone@ewha.ac.kr

Received 20 April 2007       Accepted 7 June 2007

Copyright © 2007 The Korean Academy of Tuberculosis and Respiratory Diseases

Abstract

Antiphospholipid syndrome (APS) causes recurrent thromboses and morbidity during pregnancy, including fetal loss. This malady is associated with the persistent presence of anticardiolipin antibody or lupus anticoagulant. The pulmonary manifestations of antiphospholipid syndrome include pulmonary thromboembolism, pulmonary hypertension, acute respiratory distress syndrome, etc. Pulmonary thromboembolism is often the initial manifestation of antiphospholipid syndrome and a timely diagnosis is critical due to the high mortality rate. We herein report on a 19-year-old man with pulmonary thromboembolism that was caused by primary antiphospholipid syndrome. He presented with blood-tinged sputum, fever and epigastric pain, and his chest computerized tomography showed pulmonary thromboembolism. The other possible causes of pulmonary thromboembolism were excluded and the diagnosis of primary antiphospholipid syndrome was confirmed by the lupus anticoagulant that was present on two occasions six weeks apart. We also discuss the nature and management of antiphospholipid syndrome, along with a brief review of the relevant literatures.

Keywords: Antiphospholipid syndrome, Pulmonary thromboembolism, Thrombosis

Figures and Tables

Figure 1
Chest PA on admission shows no parenchymal lesion.
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Figure 2
Chest CT scan on admission shows thrombi in the pulmonary arteries of left lower lobe anteromedial basal segment and right lower lobe posterior basal segment (left) and hemorrhagic infarction with wedge-shaped consolidation in right lower lobe posterior basal segment (right).
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Figure 3
Lung perfusion scan shows a large wedge-shaped perfusion defect in left lower lobe and another suspicious perfusion defect in right lower lobe.
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Figure 4
Chest CT scan after 9 months of anticoagulation therapy, shows small residual thrombi in left lower lobe (left) and the resolution of the previous wedge-shaped infarction in right lower lobe (right).
trd-63-72-g004

References

1. Klippel JH. Klippel JH, editor. Chapter 24. Antiphospholipid Syndrome. Primer on the Rheumatic Diseases. 2001. 12th ed. Atlanta, Georgia: Arthritis Foundation;423–426.
2. Maggiorini M, Knoblauch A, Schneider J, Russi EW. Diffuse microvascular pulmonary thrombosis associated with primary antiphospholipid antibody syndrome. Eur Respir J. 1997. 10:727–730.
3. Goldhaber SZ. Pulmonary Embolism. N Engl J Med. 1998. 339:93–104.
4. Mackworth-Young C. Primary antiphospholipid syndrome: a distinct entity? Autoimmun Rev. 2006. 5:70–75.
5. Pujol A, Alegria E, Gonzalez J, Ngare Ch, Perez J, Paramo JA. Clinical complications in a series of patients with anticardiolipin antibodies. An Sist Sanit Navar. 2003. 26:365–372.
6. Stojanovich L. Pulmonary manifestations in antiphospholipid syndrome. Autoimmun Rev. 2006. 5:344–348.
7. Cervera R, Asherson RA, Acevedo ML, Gomez-Puerta JA, Espinosa G, De La Red G, et al. Antiphospholipid syndrome associated with infections: clinical and microbiological characteristics of 100 patients. Ann Rheum Dis. 2004. 63:1312–1317.
8. Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: a systematic review. JAMA. 2006. 295:1050–1057.
9. Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003. 349:1133–1138.
10. Lockshin MD, Erkan D. Treatment of the antiphospholipid syndrome. N Engl J Med. 2003. 349:1177–1179.
11. Cherian J, Gertner E. Recurrent pulmonary embolism despite inferior vena cava filter placement in patients with the antiphospholipid syndrome. J Clin Rheumatol. 2005. 11:56–58.
12. Sandoval J, Amigo MC, Barragan R, Izaguirre R, Reyes PA, Martinez-Guerra ML, et al. Primary antiphospholipid syndrome presenting as chronic thromboembolic pulmonary hypertension. Treatment with thromboendarterectomy. J Rhumatol. 1996. 23:772–775.
13. Medina G, Vera-Lastra O, Barile L, Salas M, Jara LJ. Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients. Lupus. 2004. 13:11–16.
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