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Mediterr J Rheumatol 2016;27(4):194-7
Intracardiac thrombosis in a young female as first presentation of primary antiphospholipid syndrome: A case report
Authors Information

First Propaedeutic Department of Internal Medicine, Rheumatologic Unit, Laikon Hospital and Joint Academic Rheumatology Programme of School of Medicine, National and Kapodistrian University of Athens

Abstract
Primary antiphospholipid syndrome (PAPS) is an entity characterized by spontaneous and recurrent abortion and recurrent vascular thromboses (arterial and venous). Intracardiac thrombosis is a rare but life-threatening complication of PAPS. Herein we describe a 21 year-old woman admitted to hospital due to left pleurodynia and shortness of breath with no history of thrombotic events. Helix chest tomography scan disclosed pulmonary embolism as well as a filling defect of the inferior vena cava, which in subsequent cardiac magnetic resonance (MRI) proved to be intracardiac thrombus. Laboratory tests showed triple positivity for antiphospholipid antibodies, renal involvement and thrombocytopenia; PAPS, possibly catastrophic, was diagnosed. The patient was treated with iv pulses of corticosteroids, cyclophosphamide, intravenous immunoglobulin and oral anticoagulation (INR levels between 2.5 and 3), improved gradually and was discharged after 15 days of hospitalization. At 6-month follow-up new cardiac MRI revealed complete resolution of the thrombus. Patients with APS that present with pulmonary embolism should be investigated for the possibility of intracardial thrombus. Indefinite anticoagulation treatment in these patients is warranted due to high recurrence rates.