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Atypical haemolytic uremic syndrome with “subclinical” thrombotic microangiopathy: a single case or regularity?

Abstract

We report a case of atypical haemolytic uremic syndrome with chronic course: slow progression of chronic renal failure, resistant arterial hypertension and stable hematologic parameters during 9 years after the first manifestation. After the initiation of eculizumab therapy hypertension resolved and renal function partially improved. A new term «subclinical thrombotic microangiopathy» is suggested for the definition of this variant of aHUS. The possibility of subclinical course of thrombotic microangiopathy due to thrombotic occlusion of renal microcirculation in aHUS as well as diagnostic methods and treatment options are discussed.

About the Authors

N. L. Kozlovskaya
First Moscow State Sechenov Medical University, Moscow
Russian Federation


K. A. Demyanova
First Moscow State Sechenov Medical University, Moscow
Russian Federation


D. V. Kuznetsov
First Moscow State Sechenov Medical University, Moscow
Russian Federation


A. M. Kuchieva
First Moscow State Sechenov Medical University, Moscow
Russian Federation


L. A. Bobrova
First Moscow State Sechenov Medical University, Moscow
Russian Federation


E. S. Stolyarevich
Moscow State A.I. Evdokimov University of Medicine and Dentistry
Russian Federation


References

1. Козловская Н.Л., Шилов Е.М., Метелева Н.А и соавт. Клинико-морфологические особенности нефропатии при первичном и вторичном антифосфолипидном синдроме // Тер.архив. 2007. № 6. С. 16-25.

2. Ariceta G, Besbas N, Johnson S et al. Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome // Pediatr Nephrol. 2009. Vol. 24. P. 687-696.

3. Asif A, Haqqie SS, Ghate K et al. Continued eculizumab therapy for persistent atypical hemolytic uremic syndrome // Open Urol Nephrol J. 2013. Vol. 6. P. 44-46.

4. Campistol JM, Arias M, Ariceta G et al. An update for atypical haemolytic uremic syndrome: diagnosis and treatment // A consensus document. Nefrologia. 2013. Vol. 33. P. 27-45.

5. De Serres SA, Isenring P. Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria // Nephrol Dial Transplant. 2009. Vol. 24. P. 1048-1050.

6. Fakhouri F, Delmas Y, Provot F et al. Insights from the use in clinical practice of eculizumab in adult patients with atypical hemolytic uremic syndrome affecting the native kidneys: an analysis of 19 cases // Am J Kidney Dis. 2013. http://dx.doi.org/10.1053/j.ajkd.2013.07.11

7. Fremeaux-Bacchi V, Fakhouri F, Garnier A et al. Genetic and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults // ClinJAmSocNephrol. 2012. Vol. 8. P. 554-562.

8. Gruppo RA, Rother RP. Eculizumab for congenital atypical hemolytic-uremic syndrome // N Engl J Med. 2010. Vol. 362. P. 1746-1748.

9. Legendre CM, Licht C, Muus P. еt al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome // NEnglJMed. 2013. Vol. 368. P. 2169-2181.

10. Лора Ш., Фремю-Бачи В. Атипичный ГУС // Нефрология. 2012. Т. 16. №2. С. 16-48.

11. Nochy D., Daugas E., Droz D. et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome // J Am SocNephrol. 1999. Vol. 10. P. 507-518.

12. Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome // N Engl J Med. 2009. Vol. 361. P. 1676-1687.

13. Noris M, Caprioli J, Bresin E et al. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype // Clin J Am SocNephrol. 2010. Vol. 5. P. 1844-1859.

14. Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, haemolytic uremic syndrome, and thrombotic thrombocytopenic purpura // Kidney Int. 2001. Vol. 60. P. 831-846.

15. Taylor CM, Machin S, Wigmore SJ, Goodship TH. Clinical practice guideline for the management of atypical hemolytic uremic syndrome in the United Kingdom // BrJHaematol. 2010. Vol. 148. P. 37-47.

16. Zuber J, Le Quintrec M, Sberro-Soussan R. et al. New insights into post-renal transplant hemolytic uremic syndrome // Nat Rev Nephrol. 2011. Vol. 7. P. 23-35.

17. Zuber J, Fakhouri F, Roumenina LT et al. Use of eculizumab for atypical hemolytic uremic syndrome and C3 glomerulopathies // NatRevNephrol. 2012. Vol. 8. P. 643-657.


Review

For citations:


Kozlovskaya N.L., Demyanova K.A., Kuznetsov D.V., Kuchieva A.M., Bobrova L.A., Stolyarevich E.S. Atypical haemolytic uremic syndrome with “subclinical” thrombotic microangiopathy: a single case or regularity? Nephrology and Dialysis. 2014;16(2):280-287. (In Russ.)

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ISSN 1680-4422 (Print)
ISSN 2618-9801 (Online)