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

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.