Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome

Detalhes bibliográficos
Autor(a) principal: Cavero, T
Data de Publicação: 2019
Outros Autores: Arjona, E, Soto, K, Caravaca-Fontán, F, Rabasco, C, Bravo, L, et al.
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10400.10/2347
Resumo: 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.
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spelling Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic SyndromeAtypical hemolytic uremic syndromeEculizumabMalignant hypertensionMalignant 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.ElsevierRepositório do Hospital Prof. Doutor Fernando FonsecaCavero, TArjona, ESoto, KCaravaca-Fontán, FRabasco, CBravo, L, et al.2019-12-11T16:54:40Z2019-01-01T00:00:00Z2019-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.10/2347engKidney Int. 2019; 96 (4), 995-10041523-175510.1016/j.kint.2019.05.014metadata only accessinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2022-09-20T15:53:02Zoai:repositorio.hff.min-saude.pt:10400.10/2347Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T15:53:17.551669Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
title Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
spellingShingle Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
Cavero, T
Atypical hemolytic uremic syndrome
Eculizumab
Malignant hypertension
title_short Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
title_full Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
title_fullStr Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
title_full_unstemmed Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
title_sort Severe and Malignant Hypertension Are Common in Primary Atypical Hemolytic Uremic Syndrome
author Cavero, T
author_facet Cavero, T
Arjona, E
Soto, K
Caravaca-Fontán, F
Rabasco, C
Bravo, L, et al.
author_role author
author2 Arjona, E
Soto, K
Caravaca-Fontán, F
Rabasco, C
Bravo, L, et al.
author2_role author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório do Hospital Prof. Doutor Fernando Fonseca
dc.contributor.author.fl_str_mv Cavero, T
Arjona, E
Soto, K
Caravaca-Fontán, F
Rabasco, C
Bravo, L, et al.
dc.subject.por.fl_str_mv Atypical hemolytic uremic syndrome
Eculizumab
Malignant hypertension
topic Atypical hemolytic uremic syndrome
Eculizumab
Malignant hypertension
description 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.
publishDate 2019
dc.date.none.fl_str_mv 2019-12-11T16:54:40Z
2019-01-01T00:00:00Z
2019-01-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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dc.language.iso.fl_str_mv eng
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dc.relation.none.fl_str_mv Kidney Int. 2019; 96 (4), 995-1004
1523-1755
10.1016/j.kint.2019.05.014
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dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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