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American Journal of Kidney Diseases

Thrombotic Microangiopathy in Systemic Lupus Erythematosus: Efficacy of Eculizumab

Published:November 15, 2014DOI:https://doi.org/10.1053/j.ajkd.2014.07.031
      Thrombotic microangiopathy (TMA) is a severe disorder with poor outcomes. The cause is unknown for many patients, although TMA is associated with connective tissue disorders, including systemic lupus erythematosus (SLE). While uncommon, TMA is one of the most serious complications of SLE and in many cases may be resistant to therapy. We report a patient with SLE complicated by TMA that was refractory to standard therapy but responded well to eculizumab, with continued remission after 1 year of follow-up. Eculizumab might be useful in the management of resistant cases of TMA caused by SLE.

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      References

        • Gharbi C.
        • Bourry E.
        • Rouvier P.
        • et al.
        Rapidly progressive lupus nephritis and concomitant thrombotic microangiopathy.
        Clin Exp Nephrol. 2010; 14: 487-491
        • Hillmen P.
        • Young N.S.
        • Schubert J.
        • et al.
        The complement inhibitor eculizumab in paroxysmal nocturnal hemoglobinuria.
        N Engl J Med. 2006; 355: 1233-1243
        • Caprioli J.
        • Noris M.
        • Brioschi S.
        • et al.
        • International Registry of Recurrent and Familial HUS/TTP
        Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome.
        Blood. 2006; 108: 1267-1279
        • Wagner E.
        • Frank M.M.
        Therapeutic potential of complement modulation.
        Nat Rev Drug Discov. 2010; 1: 43-56
        • Moake J.L.
        Thrombotic microangiopathies.
        N Engl J Med. 2002; 347: 589-600
        • Veyradier A.
        • Meyer D.
        Thrombotic thrombocytopenic purpura and its diagnosis.
        J Thromb Haemost. 2005; 3: 2420-2427
        • Schmidtko J.
        • Peine S.
        • El-Housseini Y.
        • et al.
        Treatment of atypical hemolytic uremic syndrome and thrombotic microangiopathies: a focus on eculizumab.
        Am J Kidney Dis. 2013; 61: 289-299
        • Hunt B.J.
        • Tueger S.
        • Pattison J.
        • et al.
        Microangiopathic haemolytic anaemia secondary to lupus nephritis: an important differential diagnosis of thrombotic thrombocytopenic purpura.
        Lupus. 2007; 16: 358-362
        • Vaidya S.
        • Abul-ezz S.
        • Lipsmeyer E.
        Thrombotic thrombocytopenic purpura and systemic lupus erythematosis.
        Scand J Rheumatol. 2001; 30: 308-310
        • Gatenby P.A.
        • Smith H.
        • Kirwan P.
        • et al.
        Systemic lupus erythematous and thrombotic thrombocytopenic purpura: a case report and review of relationship.
        J Rheumatol. 1981; 8: 504-508
        • Blake J.S.
        • Butani L.
        Rapidly progressive lupus glomerulonephritis and concomitant microangiopathy in an adolescent.
        Lupus. 2002; 11: 533-535
        • Legendre C.M.
        • Licht C.
        • Muus P.
        • et al.
        Terminal complement inhibitor eculizumab in atypical hemolytic–uremic syndrome.
        N Engl J Med. 2013; 368: 2169-2181
        • Hadaya K.
        • Ferrari-Lacraz S.
        • Fumeaux D.
        • et al.
        Eculizumab in acute recurrence of thrombotic microangiopathy after renal transplantation.
        Am J Transplant. 2011; 11: 2523-2527
        • Barilla-Labarca M.L.
        • Toder K.
        • Furie R.
        Targeting the complement system in systemic lupus erythematosus and other diseases.
        Clin Immunol. 2013; 148: 313-321
      1. Furie R, Matis L, Rollins S, et al. A single dose, placebo-controlled, double blind, phase I study of the humanized anti-C5 antibody h5G1.1 in patients with systemic lupus erythematosus. Innovative Therapies in Autoimmune Diseases. Abstract presented at: American College of Rheumatology 68th Annual Scientific Meeting, October 19, 2004, San Antonio, TX.

        • Lonze B.E.
        • Singer A.L.
        • Montgomery R.A.
        Eculizumab and renal transplantation in a patient with CAPS.
        N Engl J Med. 2010; 362: 1744-1745
        • Shapira I.
        • Andrade D.
        • Allen S.L.
        • et al.
        Induction of sustained remission in recurrent catastrophic antiphospholipid syndrome via inhibition of terminal complement with eculizumab.
        Arthritis Rheum. 2012; 64: 2719-2723
        • Mushin S.A.
        • Khianey R.
        • Erkan D.
        Comment: discordant aPTT and anti-Xa values and outcomes in hospitalized patients treated with intravenous unfractionated heparin.
        Ann Pharmacother. 2013; 47: 1239-1240
        • Erkan D.
        • Aguiar C.L.
        • Andrade D.
        • et al.
        14th International Congress on Antiphospholipid Antibodies Task Force report on antiphospholipid syndrome treatment trends.
        Autoimmun Rev. 2014; 13: 685-696
        • Nürnberger J.
        • Philipp T.
        • Witzke O.
        • et al.
        Eculizumab for atypical hemolytic–uremic syndrome.
        N Engl J Med. 2009; 360: 542-544
        • Sinibaldi S.
        • Guzzo I.
        • Piras R.
        • et al.
        Post-transplant recurrence of atypical hemolytic uremic syndrome in a patient with thrombomodulin mutation.
        Pediatr Transplant. 2013; 17: E177-E181