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• W2314896787 abstract "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 showed diffusely congested glomeruli containing fibrin thrombi with extension of thrombi into hilar arterioles and the interstitium contained patchy acute inflammation and hemorrhage with mild to moderate interstitial fibrosis (Fig. 1A). Tubular epithelial cells showed no evidence of viral cytopathic effect, and there was no evidence of vasculitis or peritubular capillaritis. Immunofluorescence microscopy showed no glomerular or vascular staining, but highlighted focal (<10%) peritubular C4d staining. Subsequent serologies showed elevated anticardiolipin and anti-β-2 glycoprotein 1 antibodies as well as lupus anticoagulant titers. Heparin-PF4 antibody, serotonin release assay, C3/4, anti-nuclear antibody, anti-dsDNA antibodies, donor specific anti-HLA, and quantitative serologies for polyoma BK virus and cytomegalovirus were negative. A subtherapeutic tacrolimus level made tacrolimus-related TMA unlikely. Treatment with plasma exchange failed to resolve the features of TMA. Eculizumab was subsequently started with improved renal function and normalization of hemoglobin and platelets. A repeat biopsy performed after twice monthly dosing of eculizumab for 7 months demonstrated no evidence of TMA (Fig. 1B) and creatinine stabilized between 2.0 and 2.4 mg/dL. A repeat serological work-up showed no hemolysis or thrombocytopenia, normal levels of anticardiolipin, β-2-microglobulin, and lupus anticoagulant. Atypical HUS panel showed no disease-causing gene variants.FIGURE 1: (A) Glomerulus with endothelial injury and several fibrin thrombi (arrows). Jones silver 400×. (B) Glomerulus without evidence of microthrombi. Jones silver 400×.This case illustrates a number of clinically important contributions to the literature. Thrombotic microangiopathy occurred de novo 3 years after transplantation in a lupus patient with APS syndrome as opposed to nearly all reported cases occurring within 6 months (3). Additionally, our patient’s favorable prognosis is in keeping with observations by Ponticelli and Banfi (5) who showed a less severe prognosis when TMA occurred later in the posttransplant course or affected recipients of living-donors. As has been demonstrated, APS occurred independent of positive serologies for anti-dsDNA and antinuclear antibodies. As we progress to unravel the pathophysiology of APS-associated TMA, it is thus sensible to consider Eculizumab as an effective treatment first line or in cases which are resistant to plasmapheresis. Omid Bakhtar 1 Bijin Thajudeen1 Beth L. Braunhut2 Sarah E. Yost3 Erika R. Bracamonte2 Amy N. Sussman1 Bruce Kaplan4 1 Division of Nephrology University of Arizona Health Sciences Center Tucson, AZ 2 Department of Pathology University of Arizona Health Sciences Center Tucson, AZ 3 Department of Pharmacy University of Arizona Health Sciences Center Tucson, AZ 4 Center for Transplantation University of Kansas Medical Center Kansas City, KS" @default.
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• W2314896787 date "2014-08-15" @default.
• W2314896787 modified "2023-09-30" @default.
• W2314896787 title "A Case of Thrombotic Microangiopathy Associated With Antiphospholipid Antibody Syndrome Successfully Treated With Eculizumab" @default.
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• W2314896787 doi "https://doi.org/10.1097/tp.0000000000000267" @default.
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