RT Journal Article
T1 Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome.
A1 Cavero, Teresa
A1 Arjona, Emilia
A1 Soto, Karina
A1 Caravaca-Fontán, Fernando
A1 Rabasco, Cristina
A1 Bravo, Luis
A1 de la Cerda, Francisco
A1 Martín, Nadia
A1 Blasco, Miquel
A1 Ávila, Ana
A1 Huerta, Ana
A1 Cabello, Virginia
A1 Jarque, Ana
A1 Alcázar, Concepción
A1 Fulladosa, Xavier
A1 Carbayo, Javier
A1 Anaya, Sara
A1 Cobelo, Carmen
A1 Ramos, Natalia
A1 Iglesias, Elena
A1 Baltar, José
A1 Martínez-Gallardo, Rocío
A1 Pérez, Lourdes
A1 Morales, Enrique
A1 González, Roberto
A1 Macía, Manuel
A1 Draibe, Juliana
A1 Pallardó, Luis
A1 Quintana, Luis F
A1 Espinosa, Mario
A1 Barros, Xoana
A1 Pereira, Fernando
A1 Cao, Mercedes
A1 Moreno, Juan Antonio
A1 Rodríguez de Córdoba, Santiago
A1 Praga, Manuel
A1 Spanish Group for the Study of Glomerular Diseases (GLOSEN), 
K1 atypical hemolytic uremic syndrome
K1 complement
K1 eculizumab
K1 malignant hypertension
AB Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy, but pathogenic mutations in complement genes have been reported in patients with hypertension-induced thrombotic microangiopathy. Here we investigated the frequency and severity of hypertension in 55 patients with primary atypical hemolytic uremic syndrome (aHUS). A genetic analysis was performed in all patients, and funduscopic examination was performed in all the patients with Grades 2 and 3 hypertension. A cohort of 110 patients with malignant hypertension caused by diseases other than aHUS served as control. Thirty-six patients with aHUS presented Grade 2 or Grade 3 hypertension and funduscopic examination showed malignant hypertension in 19. Genetic abnormalities in complement were found in 19 patients (37% among patients with malignant hypertension). Plasmapheresis was performed in 46 patients and 26 received eculizumab. Renal and hematological responses were significantly lower after plasmapheresis (24%) than after eculizumab (81%). Renal survival was significantly higher in patients treated with eculizumab (85% at one, three and five years) compared to patients who did not receive this treatment (54%, 46% and 41%), respectively. Response to eculizumab was independent of hypertension severity and the presence of complement genetic abnormalities. Among patients with malignant hypertension caused by other diseases the prevalence of thrombotic microangiopathy was very low (5%). Thus, severe and malignant hypertension are common among patients with aHUS and eculizumab treatment leads to a higher renal survival when compared to plasmapheresis. However, thrombotic microangiopathy is uncommon among patients presenting with malignant hypertension caused by diseases other than aHUS.
YR 2019
FD 2019-05-31
LK http://hdl.handle.net/10668/14404
UL http://hdl.handle.net/10668/14404
LA en
DS RISalud
RD Apr 7, 2025