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Vol.82 No.1
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Clinical Phenotype of a First Unprovoked Acute Pulmonary Embolism Associated with Antiphospholipid Antibody Syndrome

Tuberculosis & Respiratory Diseases / Tuberculosis & Respiratory Diseases,
2019, v.82 no.1, pp.53-61






& (2019). Clinical Phenotype of a First Unprovoked Acute Pulmonary Embolism Associated with Antiphospholipid Antibody Syndrome. Tuberculosis & Respiratory Diseases, 82(1), 53-61.
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Abstract

Background: Antiphospholipid antibody syndrome (APS), an important cause of acquired thrombophilia, is diagnosed when vascular thrombosis or pregnancy morbidity occurs with persistently positive antiphospholipid antibodies (aPL). APS is a risk factor for unprovoked recurrence of pulmonary embolism (PE). Performing laboratory testing for aPL after a first unprovoked acute PE is controversial. We investigated if a specific phenotype existed in patients with unprovoked with acute PE, suggesting the need to evaluate them for APS. Methods: We retrospectively reviewed patients with PE and APS (n=24) and those with unprovoked PE with aPL negative (n=44), evaluated 2006–2016 at the Asan Medical Center. We compared patient demographics, clinical manifestations, laboratory findings, and radiological findings between the groups. Results: On multivariate logistic regression analysis, two models of independent risk factors for APS-PE were suggested. Model I included hemoptysis (odds ratio [OR], 12.897; 95% confidence interval [CI], 1.025–162.343), low PE severity index (OR, 0.948; 95% CI, 0.917–0.979), and activated partial thromboplastin time (aPTT; OR, 1.166; 95% CI, 1.040–1.307). Model II included age (OR, 0.930; 95% CI, 0.893–0.969) and aPTT (OR, 1.104; 95% CI, 1.000–1.217). Conclusion: We conclude that patients with first unprovoked PE with hemoptysis and are age <40; have a low pulmonary embolism severity index, especially in risk class I–II; and/or prolonged aPTT (above 75th percentile of the reference interval), should be suspected of having APS, and undergo laboratory testing for aPL.

keywords
Antiphospholipid Syndrome, Antibodies, Antiphospholipid, Pulmonary Embolism, Phenotype, Risk Factors

Reference

1.

1. Iorio A, Kearon C, Filippucci E, Marcucci M, Macura A, Pengo V, et al. Risk of recurrence after a first episode of symptomatic venous thromboembolism provoked by a transient risk factor:a systematic review. Arch Intern Med 2010; 170:1710-6.

2.

2. Kearon C, Akl EA. Duration of anticoagulant therapy for deep vein thrombosis and pulmonary embolism. Blood 2014; 123:1794-801.

3.

3. Konstantinides SV, Torbicki A, Agnelli G, Danchin N, Fitzmaurice D, Galie N, et al. 2014 ESC guidelines on the diagnosis and management of acute pulmonary embolism. Eur Heart J 2014; 35:3033-69, 3069a-k.

4.

4. Zhu T, Martinez I, Emmerich J. Venous thromboembolism:risk factors for recurrence. Arterioscler Thromb Vasc Biol 2009; 29:298-310.

5.

5. Kyrle PA, Rosendaal FR, Eichinger S. Risk assessment for recurrent venous thrombosis. Lancet 2010; 376:2032-9.

6.

6. Heit JA. Predicting the risk of venous thromboembolism recurrence. Am J Hematol 2012; 87 Suppl 1:S63-7.

7.

7. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4:295-306.

8.

8. Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun 2000; 15:145-51.

9.

9. Asherson RA, Cervera R. Review: antiphospholipid antibodies and the lung. J Rheumatol 1995; 22:62-6.

10.

10. Espinosa G, Cervera R, Font J, Asherson RA. The lung in the antiphospholipid syndrome. Ann Rheum Dis 2002; 61:195-8.

11.

11. Kearon C, Akl EA, Ornelas J, Blaivas A, Jimenez D, Bounameaux H, et al. Antithrombotic therapy for VTE disease: CHEST guideline and expert panel report. Chest 2016; 149:315-52.

12.

12. Mirrakhimov AE, Hill NS. Primary antiphospholipid syndrome and pulmonary hypertension. Curr Pharm Des 2014; 20:545-51.

13.

13. Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, Brey R, Crowther M, Derksen R, et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients:report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus 2011; 20:206-18.

14.

14. Galli M, Luciani D, Bertolini G, Barbui T. Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood 2003; 101:1827-32.

15.

15. Keeling D, Mackie I, Moore GW, Greer IA, Greaves M; British Committee for Standards in Haematology. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol 2012; 157:47-58.

16.

16. Pengo V, Tripodi A, Reber G, Rand JH, Ortel TL, Galli M, et al. Update of the guidelines for lupus anticoagulant detection. Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost 2009; 7:1737-40.

17.

17. Aujesky D, Obrosky DS, Stone RA, Auble TE, Perrier A, Cornuz J, et al. Derivation and validation of a prognostic model for pulmonary embolism. Am J Respir Crit Care Med 2005; 172:1041-6.

18.

18. Chan CM, Woods C, Shorr AF. The validation and reproducibility of the pulmonary embolism severity index. J Thromb Haemost 2010; 8:1509-14.

19.

19. Boyden EA. Segmental anatomy of the lungs. New York:McGraw-Hill; 1955.

20.

20. Mchrani T, Petri M. Epidemiology of the antiphospholipid syndrome. In: Asherson RA, editor. Handbook of systemic autoimmune diseases. Vol. 10. Amsterdam: Elsevier; 2009. p. 13-34.

21.

21. Gomez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun 2014; 48-49:20-5.

22.

22. Piette JC, Cacoub P. Antiphospholipid syndrome in the elderly:caution. Circulation 1998; 97:2195-6.

23.

23. Anderson FA Jr, Spencer FA. Risk factors for venous thromboembolism. Circulation 2003; 107(23 Suppl 1):I9-16.

24.

24. Pollack CV, Schreiber D, Goldhaber SZ, Slattery D, Fanikos J, O’Neil BJ, et al. Clinical characteristics, management, and outcomes of patients diagnosed with acute pulmonary embolism in the emergency department: initial report of EMPEROR (Multicenter Emergency Medicine Pulmonary Embolism in the Real World Registry). J Am Coll Cardiol 2011; 57:700-6.

25.

25. He H, Stein MW, Zalta B, Haramati LB. Pulmonary infarction:spectrum of findings on multidetector helical CT. J Thorac Imaging 2006; 21:1-7.

26.

26. Tsao MS, Schraufnagel D, Wang NS. Pathogenesis of pulmonary infarction. Am J Med 1982; 72:599-606.

27.

27. Miniati M, Bottai M, Ciccotosto C, Roberto L, Monti S. Predictors of pulmonary infarction. Medicine (Baltimore)2015; 94:e1488.

28.

28. Weng CT, Chung TJ, Liu MF, Weng MY, Lee CH, Chen JY, et al. A retrospective study of pulmonary infarction in patients with systemic lupus erythematosus from southern Taiwan. Lupus 2011; 20:876-85.

29.

29. Thrombosis and thrombocytopenia in antiphospholipid syndrome (idiopathic and secondary to SLE): first report from the Italian Registry. Italian Registry of Antiphospholipid Antibodies (IR-APA). Haematologica 1993; 78:313-8.

30.

30. Cuadrado MJ, Mujic F, Munoz E, Khamashta MA, Hughes GR. Thrombocytopenia in the antiphospholipid syndrome. Ann Rheum Dis 1997; 56:194-6.

31.

31. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46:1019-27. www.e-trd.org https://doi.org/10.4046/trd.2018.0045 61

32.

32. Uthman I, Godeau B, Taher A, Khamashta M. The hematologic manifestations of the antiphospholipid syndrome. Blood Rev 2008; 22:187-94.

33.

33. Abo SM, DeBari VA. Laboratory evaluation of the antiphospholipid syndrome. Ann Clin Lab Sci 2007; 37:3-14.

34.

34. Donze J, Le Gal G, Fine MJ, Roy PM, Sanchez O, Verschuren F, et al. Prospective validation of the Pulmonary Embolism Severity Index: a clinical prognostic model for pulmonary embolism. Thromb Haemost 2008; 100:943-8.

35.

35. Streiff MB, Agnelli G, Connors JM, Crowther M, Eichinger S, Lopes R, et al. Guidance for the treatment of deep vein thrombosis and pulmonary embolism. J Thromb Thrombolysis 2016; 41:32-67.

36.

36. Auerbach AD, Sanders GD, Hambleton J. Cost-effectiveness of testing for hypercoagulability and effects on treatment strategies in patients with deep vein thrombosis. Am J Med 2004; 116:816-28.

37.

37. Kearon C, Parpia S, Spencer FA, Baglin T, Stevens SM, Bauer KA, et al. Antiphospholipid antibodies and recurrent thrombosis after a first unprovoked venous thromboembolism. Blood 2018; 131:2151-60.

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