A case of antiphospholipid syndrome presenting with pulmonary truncus and main pulmonary artery thrombosis

Rheumatology International
Mehmet SayarliogluReha Erkoc

Abstract

In patients with antiphospholipid syndrome (APS), thromboembolism and pulmonary hypertension are the most common pulmonary manifestations. Thrombotic obstruction at the level of the main and/or proximal pulmonary arteries is rare. We report a 40-year-old woman without any history of previous arterial and/or venous thrombosis who presented with severe dyspnea and was found to have pulmonary hypertension and positivity for anticardiolipin antibodies. Computed tomography revealed pulmonary truncus thrombosis extending to both right and left pulmonary arteries. The patient and her family refused surgical treatment. She had a prolonged hospital course, was unresponsive to thrombolytic, anticoagulant, antiplatelet, and immunosuppressive treatments, and died of right ventricle and respiratory failure 5 weeks later. This is the first reported case with thrombosis of pulmonary truncus and main pulmonary arteries concurrent with APS.

References

Nov 1, 1996·The Annals of Thoracic Surgery·R S HartzS Rich
Jan 16, 1999·Baillière's Clinical Rheumatology·M C AmigoG R Hughes
Aug 6, 2000·British Journal of Haematology·M GreavesI Mackie
Apr 16, 2002·Arthritis and Rheumatism·Ricard CerveraUNKNOWN Euro-Phospholipid Project Group
Apr 4, 2003·The International Journal of Biochemistry & Cell Biology·J J Manson, D A Isenberg

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Antiphospholipid Syndrome

Antiphospholipid syndrome or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by the presence of antibodies directed against phospholipids.