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Title: An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Review
Other Titles: Actualización en síndrome hemolítico urémico atípico: diagnóstico y tratamiento. Documento de consenso. Revisión
Author: Campistol Plana, Josep M.
Arias, Manuel
Ariceta, Gema
Blasco, Miguel
Espinosa, Laura
Espinosa, Mario
Grinyo Boira, Josep M.
Macía, Manuel
Mendizábal, Santiago
Praga, Manuel
Román, Elena
Torra, Roser
Valdés, Francisco
Vilalta, Ramon (Vilalta Casas)
Rodríguez de Córdoba, Santiago
Keywords: Anèmia hemolítica
Trastorns de les plaquetes sanguínies
Insuficiència renal aguda
Malalties hematològiques
Anticossos monoclonals
Hemolytic anemia
Blood platelet disorders
Acute renal failure
Hematologic diseases
Monoclonal antibodies
Issue Date: 3-Dec-2015
Publisher: Elsevier España
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 characterises 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 complement system, which can explain approximately 60% of the aHUS cases, and a number of mutations and polymorphisms have been functionally characterised. These findings have stablished that aHUS is a consequence of the insufficient regulation of the activation 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.
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It is part of: Nefrología, 2015, vol. 35, num. 5, p. 421-447
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ISSN: 0211-6995
Appears in Collections:Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
Articles publicats en revistes (Ciències Clíniques)

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