The approaches to the diagnosis and treatment of atypical hemolytic uremic syndrome in the practice of a nephrologist


Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

The article presents insights into the diagnosis and treatment of secondary thrombotic microangiopathy (ТМА) and the specific features of differential diagnosis of aHUS from other forms of ТМА. Contemporary principles of aHUS management and genetic characteristics of the disease are discussed in details.

Full Text

Restricted Access

References

  1. Goodship T., Cook H.T., Fakhouri F., Fervenza F.C., Frémeaux-Bacchi V., Kavanagh D., Nester C.M., Noris M., Pickering M.C., Rodriguez de Cordoba S., Roumenina L.T., Sethi S., Smith R.J.; Conference Participants. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a «Kidney Disease: Improving Global Outcomes» (KDIGO) Controversies Conference. Kidney Int. 2017; 91(3): 539-551. doi: 10.1016/j.kint.2016.10.005.
  2. Клинические рекомендации по диагностике и лечению атипичного гемолитико-уремического синдрома. Нефрология и диализ. 2015; 17(3): 242-264.
  3. Козловская Н.Л., Демьянова К.А., Тутер Д.С., Щекочихин Д.Ю., Копылов Ф.Ю., Бондаренко Т.В., Рощупкина С.В., Шилов Е.М., Сыркин А.Л. Поражение сердца у больных атипичным гемолитико-уремическим синдромом. Клиническая нефрология. 2015; 5-6: 32-38.
  4. Walle J.V., Delmas Y., Ardissino G., Wang J., Kincaid J.F., Haller H. Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J. Nephrol. 2016; 30(1): 127-134. doi: 10.1007/s40620-016-0288-3.
  5. Fakhouri F., Hourmant M., Campistol J.M., Cataland S.R., Espinosa M., Gaber A.O., Menne J., Minetti E.E., Provôt F., Rondeau E., Ruggenenti P., Weekers L.E., Ogawa M., Bedrosian C.L., Legendre C.M. Terminal complement inhibitor eculizumab in adult patients with atypical hemolytic uremic syndrome: a single-arm, open-label trial. AJKD. 2016; 68(1): 84-93. doi: 10.1053/j.ajkd.2015.12.034.
  6. Asif A., Nayer A., Haas C.S. Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab. J. Nephrol. 2016; 15.
  7. Laurence J., Haller H., Mannucci P.M., Nangaku M., Praga M., Rodriguez de Cordoba S. Atypical Hemolytic Uremic Syndrome (aHUS): Essential Aspects of an Accurate Diagnosis. Clin. Adv. Hematol. Oncol. 2016; 11(11): 2-15.
  8. Campistol J.M., Arias M., Ariceta G., Blasco M., Espinosa L., Espinosa M., Grinyo J.M., Macia M., Mendizdbal S., Praga M., Roman E., Torra R., Valdés F., Vilalta R., Rodriguez de Cordoba S. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia. 2015; 35(5): 421-447. doi: 10.1016/j.nefro.2015.07.005.
  9. Noris M., Mescia F., Remuzzi G. STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat. Rev. Nephrol. 2012; 8(11): 622-633. doi: 10.1038/nrneph.2012.195.
  10. Mitra D., Jaffe E.A., Weksler B., Hajjar K.A., Soderland C., Laurence J. Thrombotic thrombocytopenic purpura and sporadic hemolytic-uremic syndrome plasmas induce apoptosis in restricted lineages of human microvascular endothelial cells. Blood. 1997; 89(4): 1224-1234.
  11. Hofer J., Rosales A., Fischer C., Giner T. Extra-renal manifestations of complement-mediated thrombotic microangiopathies. Front. Pediatr. 2014; 2: 97. doi: 10.3389/fped.2014.00097.
  12. Zuber J., Fakhouri F., Roumenina L. T., Loirat C., Frémeaux-Bacchi V. French Study Group for aHUS/C3G. Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat. Rev. Nephrol. 2012; 8(11): 643-657. doi: 10.1038/nrneph.2012.214.
  13. Licht C., et al. Eculizumab (ECU) safety and efficacy in atypical hemolytic uremic syndrome (aHUS) patients with long disease duration and chronic kidney disease (CKD): 2-year results [ASH abstract 985]. Blood. 2012; 120(21).
  14. Chapin J., Weksler B., Magro C., Laurence J. Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura. Br. J. Haematol. 2012; 157(6): 772-774. doi: 10.1111/j.1365-2141.2012.09084.x.
  15. Magro C.M., Momtahen S., Mulvey J.J., Yassin A.H., Kaplan R.B., Laurence J. C. Role of the skin biopsy in the diagnosis of atypical hemolytic uremic syndrome. Am. J. Dermatopathol. 2015; 37(5): 349-356. doi: 10.1097/DAD.0000000000000234.
  16. Ohanian M., Cable C., Halka K. Eculizumab safely reverses neurologic impairment and eliminates need for dialysis in severe atypical hemolytic uremic syndrome. Clin. Pharmacol. 2011; 3: 5-12. doi: 10.2147/CPAA.S17904.
  17. Reti M., Farkas P., Csuka D., Râzso K., Schlammadinger Â., Udvardy M.L., Madâch K., Domjân G., Bereczki C., Reusz G.S., Szabo A.J., Prohâszka Z. Complement activation in thrombotic thrombocytopenic purpura. J. Thromb. Haemost. 2012; 10(5): 791-798. doi: 10.1111/j.1538-7836.2012.04674.x.
  18. Noris M., Caprioli J., Bresin E., Mossali C., Pianetti G., Gamba S., Daina E., Fenili C., Castelletti F., Sorosina A., Piras R., Donadelli R., Maranta R., van der Meer I., Conway E.M., Zipfel P.F., Goodship T.H., Remuzzi G. Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype. Clin. J. Am. Soc. Nephrol. 2010; 5(10): 1844-1859. doi: 10.2215/CJN.02210310.
  19. Fakhouri F., Hourmant M., Campistol J.M., et al. Eculizumab inhibits thrombotic microangiopathy and improves renal function in adult atypical hemolytic uremic syndrome patients: 1-year update. American Society of Nephrology. Philadelphia, 2014. Abstract SA-PO508.
  20. Coppo R., Peruzzi L., Amore A., Martino S., Vergano L., Lastauka I., Schieppati A., Noris M., Tovo P.A., Remuzzi G. Dramatic efects of eculizumab in a child with difuse proliferative lupus nephritis resistant to conventional therapy. Pediatr. Nephrol. 2015; 30: 167-172. doi: 10.1007/s00467-014-2944-y.
  21. El-Husseini A., Hannan S., Awad A., Jennings S., Cornea V., Sawaya B.P. Thrombotic microangiopathy in systemic lupus erythematosus: eicacy of eculizumab. Am. J. Kidney Dis. 2015; 65: 127-130. doi: 10.1053/j.ajkd.2014.07.031.
  22. Hadaya K., Ferrari-Lacraz S., Fumeaux D., Boehlen F., Toso C., Moll S., Martin P.Y., Villard J. Eculizumab in acute recurrence of thrombotic microangiopathy after renal transplantation. Am. J. Transplant. 2011; 11: 2523-2527. doi: 10.1111/j.1600-6143.2011.03696.x.
  23. Ohta T., Urayama K., Tada Y., Furue T., Imai S., Matsubara K., Ono H., Sakano T., Jinno K., Yoshida Y., Miyata T., Fujimura Y. Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension. Pediatr. Nephrol. 2015; 30: 603-608. doi: 10.1007/s00467-014-2975-4.
  24. Licht C., Ardissino G., Ariceta G., Cohen D., Cole J.A., Gasteyger C., Greenbaum L.A., Johnson S., Ogawa M., Schaefer F., Vande Walle J., Frémeaux-Bacchi V. The global aHUS registry: methodology and initial patient characteristics. BMC Nephrology. 2015; 16: 207. doi: 10.1186/s12882-015-0195-1.
  25. Бюллетень Федеральной службы государственной статистики. URL: http://www.gks.ru
  26. Бюллетень ТО Федеральной службы государственной статистики по г. Москве. URL: http://moscow.gks.ru

Supplementary files

Supplementary Files
Action
1. JATS XML

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies