ABSTRACT We report a 14-year-old patient with recurrent pulmonary embolism due to catastrophic antiphospholipid syndrome (APS) with severe pulmonary inflammation. We considered elevated antibodies against cardiolipin and anti-beta2-glycoprotein-1, but no clinical nor laboratory manifestations of systemic lupus erythematosus (SLE). Pulmonary embolism had been the first manifestation of catastrophic APS in this patient. We prescribed warfarin and systemic corticosteroids. A second embolism appeared during anticoagulation with warfarin. This event led to a marked decrease of his physical performance due to his obstructive and restrictive lung disease. Anticoagulation was changed from enteral warfarin to subcutaneous enoxaparine. We also prescribed inhaled corticosteroid which led to an improvement of his respiratory symptoms and overall poor physical condition.
Cite this paper
Müller, C. , Willaschek, C. , Borst, M. and Buchhorn, R. (2013) Recurrent pulmonary embolism in a boy with antiphospholipid syndrome. Case Reports in Clinical Medicine, 2, 310-314. doi: 10.4236/crcm.2013.25083.
 von Scheven, E., Glidden, D.V. and Elder, M.E. (2002) Anti-beta2-glycoprotein I antibodies in pediatric systemic lupus erythematosus and antiphospholipid syndrome. Arthritis & Rheumatism, 47, 414-420.
 Espinosa, G., Cervera, R., Font, J. and Asherson, R.A. (2002) The lung in the antiphospholipid syndrome. Annals of the Rheumatic Diseases, 61, 195-198.
 Asherson, R.A., et al. (1998) Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore), 77, 195-207.
 Asherson, R.A. and Cervera, R. (1995) Review: Antiphospholipid antibodies and the lung. Journal of Rheumatology, 22, 62-66.
 Cervera, R., et al. (2009) Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: A multicenter prospective study of 1,000 patients. Annals of the Rheumatic Diseases, 68, 1428-1432.
 Olivier, C., Blondiaux, E., Blanc, T., Borg, J.Y. and Dacher, J.N. (2006) Catastrophic antiphospholipid syndrome and pulmonary embolism in a 3-year-old child. Pediatric Radiology, 36, 870-873. doi:10.1007/s00247-006-0183-5
 Porres-Aguilar, M., Pena-Ruiz, M.A., Burgos, J.D., Porres-Munoz, M. and Hughes, H.W. (2008) Chronic thromboembolic pulmonary hypertension as an uncommon presentation of primary antiphospholipid syndrome. Journal of the National Medical Association, 100, 734-736.
 Espinosa, G., Bucciarelli, S., Asherson, R.A. and Cervera, R. (2008) Morbidity and mortality in the catastrophic antiphospholipid syndrome: Pathophysiology, causes of death, and prognostic factors. Seminars in Thrombosis and Hemostasis, 34, 290-294. doi:10.1055/s-0028-1082274
 Tan, E.M., et al. (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatism, 25, 1271-1277.
 Hochberg, M.C. (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatism, 40, 1725. doi:10.1002/art.1780400928
 Petri, M. (2005) Review of classification criteria for systemic lupus erythematosus. Rheumatic Disease Clinics of North America, 31, 245-254.
 Gaubitz, M. and Schotte, H. (2005) Early diagnosis in patients with systemic lupus erythematosus (SLE). Zeitschrift für Rheumatologie, 64, 547-552.
 Gertner, E. and Lie, J.T. (1993) Pulmonary capillaritis, alveolar hemorrhage, and recurrent microvascular thrombosis in primary antiphospholipid syndrome. Journal of Rheumatology, 20, 1224-1228.
 Lim, W., Crowther, M.A. and Eikelboom, J.W. (2006) Management of antiphospholipid antibody syndrome: A systematic review. JAMA, 295, 1050-1057.
 Crowther, M.A., et al. (2003) A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. New England Journal of Medicine, 349, 1133-1138. doi:10.1056/NEJMoa035241
 Ruiz-Irastorza, G., Hunt, B.J. and Khamashta, M.A. (2007) A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis & Rheumatism, 57, 1487-1495. doi:10.1002/art.23109
 Castro, P., et al. (2003) Catastrophic antiphospholipid syndrome and acute heart failure. Report of a case. Revista Medica de Chile, 131, 1037-1041.
 Huang, H.J., Wang, J.N., Tsai, Y.C., Lin, C.S. and Wu, J.M. (2002) Intrapulmonary thrombolytic therapy in a child with acute pulmonary embolism due to primary antiphospholipid syndrome. Acta Paediatrica Taiwanica, 43, 351-353.
 Kim, W.J., Hong, Y.K. and Yoo, W.H. (2008) Epidural hematoma mimicking transverse myelitis in a patient with primary antiphospholipid syndrome. Rheumatology International, 28, 709-712. doi:10.1007/s00296-007-0512-5
 Ordi-Ros, J., Perez-Peman, P. and Monasterio, J. (1994) Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies). Haemostasis, 24, 165-174.
 Cherian, J. and Gertner, E. (2005) Recurrent pulmonary embolism despite inferior vena cava filter placement in patients with the antiphospholipid syndrome. Journal of Clinical Rheumatology, 11, 56-58.
 Zifman, E., Rotman-Pikielny, P., Berlin, T. and Levy, Y. (2009) Insertion of inferior vena cava filters in patients with the antiphospholipid syndrome. Seminars in Arthritis and Rheumatism, 38, 472-477.
 Palareti, G., et al. (2006) D-dimer testing to determine the duration of anticoagulationtherapy. New England Journal of Medicine, 355, 1780-1789.