Primary antiphospholipid syndrome with refractory alveolar hemorrhage and severe mitral regurgitation: a case report and literature review

Abstract

The antiphospholipid syndrome (APS) is a hematological disorder that presents a state of hypercoagulability of autoimmune origin, characterized by thrombotic venous and / or arterial thrombosis, recurrent fetal loss and antiphospholipid antibodies presence. Another manifestation of this disease is diffuse alveolar hemorrhage, clinical entity that may occur with adult respiratory distress syndrome, respiratory failure and high mortality. Among its causes, we find lupus erythematosus, Behcet’s disease, microscopic polyarteritis, cryoglobulinemia vasculitis, Goodpasture’s syndrome, and granulomatous vasculitis. The differential diagnosis in a patient with severe valvular disease secondary to APS is a clinical challenge. We report a case of a 31- year-old woman, diagnosed with primary antiphospholipid syndrome with severe mitral regurgitation, who presented three episodes of diffuse alveolar hemorrhage, refractory to medical management. The case is discussed and a literature review is performed.