title: Early treatment with eculizumab in atypical haemolytic uraemic syndrome.
abstract: Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysis, normalize thrombocyte levels and increase diuresis. Full recovery of renal function was not possible due to irreversible renal damage prior to eculizumab initiation. These findings highlight the importance of early treatment with eculizumab in patients with poor response to standard therapy, in order to avoid irreversible renal damage.
authors.affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.
authors.forename: Maria
authors.initials: M
authors.lastname: Garjau
authors.affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.
authors.forename: María
authors.initials: M
authors.lastname: Azancot
authors.affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.
authors.forename: Rosa
authors.initials: R
authors.lastname: Ramos
authors.affiliation: Unidad de Investigacio´n, Hospital La Paz, Madrid, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Sánchez-Corral
authors.affiliation: Servicio de Anatomía Patológica, Hospital Vall d´Hebrón, Barcelona, Spain.
authors.forename: Maria Angeles
authors.initials: MA
authors.lastname: Montero
authors.affiliation: Servicio de Nefrología, Hospital Vall d´Hebrón, Barcelona, Spain.
authors.forename: Daniel
authors.initials: D
authors.lastname: Serón
keywords: atypical haemolytic uraemic syndrome; complement system; eculizumab; plasmaphaeresis-resistant