A Case of Thrombotic Microangiopathy Associated With Antiphospholipid Antibody Syndrome Successfully Treated With Eculizumab

作者：Omid R. Bakhtar, Bijin Thajudeen, Beth L. Braunhut, Sarah E. Yost, Erika R. Bracamonte, Amy N. Sussman, Bruce E. Kaplan · 发表于：Transplantation · 年份：2014 · DOI：10.1097/tp.0000000000000267 · 被引用次数：28 · 研究领域：Systemic Lupus Erythematosus Research、Renal Diseases and Glomerulopathies、Vasculitis and related conditions

A case of thrombotic microangiopathy (TMA) associated with antiphospholipid antibody syndrome (APS) successfully treated with eculizumab. Antiphospholipid antibody syndrome is a multisystem disorder characterized by vascular thrombosis and presence of circulating autoantibodies. The presence of APS can predispose to macrovascular thrombotic events, such as allograft thrombosis after kidney transplantation as well as microvascular thrombotic renal disease (1) and is associated with a poor functional outcome in the first posttransplant year (2). Recent data have demonstrated the involvement of complement pathway in the pathogenesis of TMA because of APS nephropathy (3) and prevention (4) with a complement C5 inhibitor has been found to be effective. We describe a case of TMA secondary to APS successfully treated with eculizumab. A 26-year-old man was admitted with abdominal discomfort, vomiting, and black stools lasting 3 days. His medical history was significant for class V lupus nephritis with a living related kidney transplant 3 years prior and Libman-Sacks endocarditis status post mitral valve replacement. His immunosuppression comprised of thymoglobulin induction and maintenance with tacrolimus, mycophenolate, and prednisone. Physical examination was only remarkable for epigastric tenderness. Two days after admission, there was a fall in hemoglobin and platelets with simultaneous elevation in creatinine and significant evidence of intravascular hemolysis. A renal biopsy sh...