Pediatr Allergy Respir Dis.  2005 Sep;15(3):316-323.

A Case of Antiphospholipid Syndrome Presenting as Pulmonary Infarction and Multiple Arterial Thromboembolism

• Koo SE
• Jang JY
• Lim YJ
• Lee SY
• Kim HB
• Hong SJ

Abstract

Antiphospholipid syndrome (APS) is a noninflammatory autoimmune disorder characterized by the association of arterial and/or venous thrombosis, recurrent fetal loss and elevated titres of antiphospholipid antibodies, namely lupus anticoagulant (LAC) and/or anticardiolipin antibodies (aCL). It can either occur as a free-standing condition (primary APS) or be associated with another autoimmune disease (secondary APS), mainly systemic lupus erythematosus. The precise pathogenesis of thrombosis in APS in unknown. For children with unexplainable venous or arterial thrombosis, APS should be considered. The diagnosis of APS in children requires a clinical event including venous or arterial thrombosis or immune thrombocytopenia and a laboratory abnormality including positive LAC test or positive aCL antibody test, moderate or high titer IgG. The laboratory abnormality should persist for at least 2 months. We report a 7-year-old girl who had pulmonary embolism and multiple arterial thrombosis caused by primary APS. The laboratory abnormality was positive LAC and beta2-glycoprotein I. She was treated successfully with low molecualr weight heparin (LMWH). After recovery, she was continuously treated with subcutaneous LMWH for another 3 months and switched to oral warfarin. She was symptom-free through 3 years of follow-up.

Keyword

Antiphospholipid syndrome; Child; Pulmonary embolism

MeSH Terms

Antibodies, Anticardiolipin
Antibodies, Antiphospholipid
Antiphospholipid Syndrome*
Autoimmune Diseases
beta 2-Glycoprotein I
Child
Diagnosis
Female
Follow-Up Studies
Heparin
Heparin, Low-Molecular-Weight
Humans
Immunoglobulin G
Lupus Coagulation Inhibitor
Lupus Erythematosus, Systemic
Pulmonary Embolism
Pulmonary Infarction*
Thrombocytopenia
Thromboembolism*
Thrombosis
Venous Thrombosis
Warfarin
Antibodies, Anticardiolipin
Antibodies, Antiphospholipid
Heparin
Heparin, Low-Molecular-Weight
Immunoglobulin G
Lupus Coagulation Inhibitor
Warfarin
beta 2-Glycoprotein I