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An update for atypical haemolytic uraemic syndrome: Diagnosis and treatment. A consensus document

Data de publicació:

Autors de IIS La Fe

Autors aliens a IIS La Fe

  • Campistol, JM
  • Arias, M
  • Ariceta, G
  • Blasco, M
  • Espinosa, L
  • Espinosa, M
  • Grinyo, JM
  • Macia, M
  • Praga, M
  • Torra, R
  • Valdes, F
  • Vilalta, R
  • de Cordoba, SR

Abstract

Haemolytic uraemic syndrome (HUS) is a clinical entity defined as the triad of nonimmune haemolytic anaemia, thrombocytopenia, and acute renal failure, in which the underlying lesions are mediated by systemic thrombotic microangiopathy (TMA). Different causes can induce the TMA process that characterizes HUS. In this document we consider atypical HUS (aHUS) a sub-type of HUS in which the TMA phenomena are the consequence of the endotelial damage in the microvasculature of the kidneys and other organs due to a disregulation of the activity of the complement system. In recent years, a variety of aHUs-related mutations have been identified in genes of the the complement system, which can explain approximately 60% of the aHUS cases, and a number of mutations and polymorphisms have been functionally characterized. These findings have stablished that aHUS is a consequence of the insufficient regulation of the activiation of the complement on cell surfaces, leading to endotelial damage mediated by C5 and the complement terminal pathway. Eculizumab is a monoclonal antibody that inhibits the activation of C5 and blocks the generation of the pro-inflammatory molecule C5a and the formation of the cell membrane attack complex. In prospective studies in patients with aHUS, the use of Eculizumab has shown a fast and sustained interruption of the TMA process and it has been associated with significative long-term improvements in renal function, the interruption of plasma therapy and important reductions in the need of dialysis. According to the existing literature and the accumulated clinical experience, the Spanish aHUS Group published a consensus document with recommendations for the treatment of aHUs (Nefrologia 2013;33[1]:27-45). In the current online version of this document, we update the aetiological classification of TMAs, the pathophysiology of aHUS, its differential diagnosis and its therapeutic management. (C) 2015 Sociedad Espanola de Nefrologia. Published by Elsevier Espana, S.L.U.

Dades de la publicació

ISSN/ISSNe:
0211-6995, 1989-2284

NEFROLOGIA  SOC ESPANOLA NEFROLOGIA DR RAFAEL MATESANZ

Tipus:
Review
Pàgines:
421-447
PubMed:
26456110
Factor d'Impacte:
0,437 SCImago ℠
Quartil:
Q3 SCImago ℠

Cites Rebudes en Web of Science: 121

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Keywords

  • Atypical haemolytic uraemic; syndrome; Eculizumab; Complement; Thrombotic microangiopathy

Projectes associats

REGISTRO NACIONAL DE RECHAZO HUMORAL: ESTUDIO EPIDEMIOLOGICO MULTICENTRICO OBSERVACIONAL PROSPECTIVO PARA EVALUAR LAS CARACTERISTICAS CLINICAS, SEROLOGICAS E HISTOLOGICAS Y LA EVOLUCION A 5 AÑOS DEL RECHAZO HUMORAL POST-TRASPLANTE RENAL EN ESPAÑA

Investigador Principal: ISABEL BENEYTO CASTELLO

HUMORAL-KTX . 2011

UN ESTUDIO OBSERVACIONAL, NO INTERVENCIONISTA, MULTICÉNTRICO Y MULTINACIONAL DE PACIENTES CON SÍNDROME HEMOLÍTICO URÉMICO ATÍPICO (REGISTRO DE SHUA).

Investigador Principal: ELENA ROMÁN ORTIZ

ALE-ECU-2012-01 . 2013

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