American Journal of Kidney Diseases
Volume 55, Issue 5 , Pages 923-927 , May 2010

Pulse Cyclophosphamide Therapy and Clinical Remission in Atypical Hemolytic Uremic Syndrome With Anti–Complement Factor H Autoantibodies

  • Olivia Boyer, MD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France
    • Corresponding Author InformationAddress correspondence to Olivia Boyer, MD, Service de Néphrologie Pédiatrique, Hôpital Necker Enfants Malades, 149 rue de Sèvres, 75743 Paris cedex 15, France
    • ,
  • Eve Balzamo, MD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France
    • ,
  • Marina Charbit, MD, PhD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France
    • ,
  • Nathalie Biebuyck-Gougé, MD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France
    • ,
  • Rémi Salomon, MD, PhD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France
    • ,
  • Marie-Agnès Dragon-Durey, MD, PhD

      Affiliations

    • Laboratoire d'Immunologie, Hôpital Européen Georges Pompidou, APHP, Université Paris Descartes, Paris, France
    • ,
  • Véronique Frémeaux-Bacchi, MD, PhD

      Affiliations

    • Laboratoire d'Immunologie, Hôpital Européen Georges Pompidou, APHP, Université Paris Descartes, Paris, France
    • ,
  • Patrick Niaudet, MD

      Affiliations

    • Néphrologie Pédiatrique et centre de référence MARHEA, Hôpital Necker-Enfants Malades, APHP, Université Paris Descartes, Paris, France

Received 5 September 2009 ,Accepted 22 December 2009.

References 

  1. Dragon-Durey MA, Fremeaux-Bacchi V. Atypical haemolytic uraemic syndrome and mutations in complement regulator genes. Springer Semin Immunopathol. 2005;27(3):359–374
  2. Saunders RE, Abarrategui-Garrido C, Fremeaux-Bacchi V, et al. The interactive factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and factor I mutations with structural models. Hum Mutat. 2007;28(3):222–234
  3. Caprioli J, Noris M, Brioschi S, et al. Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood. 2006;108(4):1267–1279
  4. Dragon-Durey MA, Fremeaux-Bacchi V, Loirat C, et al. Heterozygous and homozygous factor H deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol. 2004;15(3):787–795
  5. Jokiranta TS, Jaakola VP, Lehtinen MJ, Parepalo M, Meri S, Goldman A. Structure of complement factor H carboxyl-terminus reveals molecular basis of atypical haemolytic uremic syndrome. EMBO J. 2006;25(8):1784–1794
  6. Dragon-Durey MA, Loirat C, Cloarec S, et al. Anti-factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2005;16(2):555–563
  7. Dragon-Durey MA, Blanc C, Marliot F, et al. The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome. J Med Genet. 2009;46(7):447–450
  8. Kwon T, Belot A, Ranchin B, et al. Varicella as a trigger of atypical haemolytic uraemic syndrome associated with complement dysfunction: two cases. Nephrol Dial Transplant. 2009;24(9):2752–2754
  9. Kwon T, Dragon-Durey MA, Macher MA, et al. Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome. Nephrol Dial Transplant. 2008;23(6):2088–2090
  10. Le Quintrec M, Zuber J, Noel LH, et al. Anti-factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome. Am J Transplant. 2009;9(5):1223–1229
  11. Lee BH, Kwak SH, Shin JI, et al. Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency. Pediatr Res. 2009;66(3):336–340
  12. Skerka C, Jozsi M, Zipfel PF, Dragon-Durey MA, Fremeaux-Bacchi V. Autoantibodies in haemolytic uraemic syndrome (HUS). Thromb Haemost. 2009;101(2):227–232
  13. Jozsi M, Licht C, Strobel S, et al. Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency. Blood. 2008;111(3):1512–1514
  14. Jozsi M, Strobel S, Dahse HM, et al. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood. 2007;110(5):1516–1518
  15. Zipfel PF, Edey M, Heinen S, et al. Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome. PLoS Genet. 2007;3(3):e41
  16. Fakhouri F, Vernant JP, Veyradier A, et al. Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood. 2005;106(6):1932–1937
  17. Bresin E, Gastoldi S, Daina E, et al. Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies. Thromb Haemost. 2009;101(2):233–238
  18. Appel GB, Contreras G, Dooley MA, et al. Mycophenolate mofetil versus cyclophosphamide for induction treatment of lupus nephritis. J Am Soc Nephrol. 2009;20(5):1103–1112

 Originally published online as doi:10.1053/j.ajkd.2009.12.026 on March 4, 2010.

PII: S0272-6386(09)01664-3

doi: 10.1053/j.ajkd.2009.12.026

American Journal of Kidney Diseases
Volume 55, Issue 5 , Pages 923-927 , May 2010

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