"Possible primary antiphospholipid syndrome" with concurrent diffuse alveolar hemorrhaging and libman-sacks endocarditis mimicking catastrophic antiphospholipid syndrome

Abstract

Although antiphospholipid antibody syndrome (APS) is an autoimmune condition that is primarily characterized by arterial or venous thrombosis or pregnancy morbidity and the presence of antiphospholipid antibodies (aPL), recent reviews have introduced non-thromboembolic manifestations. We describe the case of a 58-year-old woman with vegetation on the aortic valve, whose initial presentation of APS abruptly developed into diffuse pulmonary hemorrhage. Despite consecutive plasma exchange procedures and the administration of corticosteroids and high-dose intravenous immunoglobulin, multiple brain infarctions developed, and the patient died of pneumonia. Although anecdotal, this case might serve as a useful example of the non-standard complications of fulminant APS. © 2012 The Japanese Society of Internal Medicine.