RT Journal Article
T1 Eculizumab in secondary atypical haemolytic uraemic syndrome.
A1 Cavero, Teresa
A1 Rabasco, Cristina
A1 Lopez, Antia
A1 Roman, Elena
A1 Avila, Ana
A1 Sevillano, Angel
A1 Huerta, Ana
A1 Rojas-Rivera, Jorge
A1 Fuentes, Carolina
A1 Blasco, Miquel
A1 Jarque, Ana
A1 Garcia, Alba
A1 Mendizabal, Santiago
A1 Gavela, Eva
A1 Macia, Manuel
A1 Quintana, Luis F
A1 Romera, Ana Maria
A1 Borrego, Josefa
A1 Arjona, Emi
A1 Espinosa, Mario
A1 Portoles, Jose
A1 Gracia-Iguacel, Carolina
A1 Gonzalez-Parra, Emilio
A1 Aljama, Pedro
A1 Morales, Enrique
A1 Cao, Mercedes
A1 Rodriguez-de-Cordoba, Santiago
A1 Praga, Manuel
K1 Atypical haemolytic uraemic syndrome
K1 Complement activation
K1 Eculizumab
K1 Thrombotic microangiopathies
AB Complement dysregulation occurs in thrombotic microangiopathies (TMAs) other than primary atypical haemolytic uraemic syndrome (aHUS). A few of these patients have been reported previously to be successfully treated with eculizumab. We identified 29 patients with so-called secondary aHUS who had received eculizumab at 11 Spanish nephrology centres. Primary outcome was TMA resolution, defined by a normalization of platelet count (>150 × 10 9 /L) and haemoglobin, disappearance of all the markers of microangiopathic haemolytic anaemia (MAHA), and improvement of renal function, with a ≥25% reduction of serum creatinine from the onset of eculizumab administration. Twenty-nine patients with secondary aHUS (15 drug-induced, 8 associated with systemic diseases, 2 with postpartum, 2 with cancer-related, 1 associated with acute humoral rejection and 1 with intestinal lymphangiectasia) were included in this study. The reason to initiate eculizumab treatment was worsening of renal function and persistence of TMA despite treatment of the TMA cause and plasmapheresis. All patients showed severe MAHA and renal function impairment (14 requiring dialysis) prior to eculizumab treatment and 11 presented severe extrarenal manifestations. A rapid resolution of the TMA was observed in 20 patients (68%), 15 of them showing a ≥50% serum creatinine reduction at the last follow-up. Comprehensive genetic and molecular studies in 22 patients identified complement pathogenic variants in only 2 patients. With these two exceptions, eculizumab was discontinued, after a median of 8 weeks of treatment, without the occurrence of aHUS relapses. Short treatment with eculizumab can result in a rapid improvement of patients with secondary aHUS in whom TMA has persisted and renal function worsened despite treatment of the TMA-inducing condition.
PB Oxford University Press
YR 2017
FD 2017-02-20
LK http://hdl.handle.net/10668/11000
UL http://hdl.handle.net/10668/11000
LA en
NO Cavero T, Rabasco C, López A, Román E, Ávila A, Sevillano Á, et al. Eculizumab in secondary atypical haemolytic uraemic syndrome. Nephrol Dial Transplant. 2017 Mar 1;32(3):466-474
NO Work in this report was funded by the Instituto de Salud Carlos III: REDinREN (RD 016/009 Feder Funds), the Fondo de Investigaciones Sanitarias (13/02502 and ICI14/00350), the Ministerio de Economia y Competitividad (SAF2015-66287R) and the Autonomous Region of Madrid (S2010/BMD-2316;Grupo de Investigacion Complemento-CM). SRdeC is funded by the Seventh Framework Programme European Union Project EURenOmics (305608).
DS RISalud
RD Aug 1, 2025