Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003 |
Resumo: | Background: In children with idiopathic nephrotic syndrome, rituximab can maintain short-term remission allowing steroids and calcineurin inhibitors taper. Methods: We retrospectively reviewed all children receiving rituximab for idiopathic nephrotic syndrome at a tertiary hospital. All patients, except one who received a single dose, had four infusions of 375mg/m2 of rituximab at a one-week interval, minimum follow-up duration of four and maximum 118 months. Results: Eleven patients were included, three patients with steroid resistant nephrotic syndrome and eight steroid dependent or frequently relapsing nephrotic syndrome. Male:female ratio was 8:3 and median age at onset was 2.7 (minimum 1.7-maximum 9.9) years-old. All patients received a trial of calcineurin inhibitors, eight received cyclophosphamide and seven mycophenolate mofetil. Remission of proteinuria was observed in nine patients. Two patients were non-responders evolving to end-stage renal disease. Patients experienced in median three relapses during the six months preceding rituximab. Responders did not relapse in the following six months (p=0.013) and daily dose of prednisolone was significantly reduced (p=0.001). Two patients relapsed at 9 and 12 months after rituximab. Another patient experienced relapse immediately after kidney transplant, successfully treated with our protocol for focal and segmental glomerulosclerosis relapse. Recovery of CD19 cells counting was observed in six patients including two relapsing patients. No severe infection or neoplasia were reported. Conclusions: Rituximab successfully reduced relapsing in complicated idiopathic nephrotic syndrome, steroid dose and enabled other immunosuppressants weaning off in most patients. Recovery of CD19 cells did not anticipate relapse, but may herald the need for additional rituximab infusions. |
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Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experiencenephrotic syndromesteroid-dependentsteroid-resistantrituximabBackground: In children with idiopathic nephrotic syndrome, rituximab can maintain short-term remission allowing steroids and calcineurin inhibitors taper. Methods: We retrospectively reviewed all children receiving rituximab for idiopathic nephrotic syndrome at a tertiary hospital. All patients, except one who received a single dose, had four infusions of 375mg/m2 of rituximab at a one-week interval, minimum follow-up duration of four and maximum 118 months. Results: Eleven patients were included, three patients with steroid resistant nephrotic syndrome and eight steroid dependent or frequently relapsing nephrotic syndrome. Male:female ratio was 8:3 and median age at onset was 2.7 (minimum 1.7-maximum 9.9) years-old. All patients received a trial of calcineurin inhibitors, eight received cyclophosphamide and seven mycophenolate mofetil. Remission of proteinuria was observed in nine patients. Two patients were non-responders evolving to end-stage renal disease. Patients experienced in median three relapses during the six months preceding rituximab. Responders did not relapse in the following six months (p=0.013) and daily dose of prednisolone was significantly reduced (p=0.001). Two patients relapsed at 9 and 12 months after rituximab. Another patient experienced relapse immediately after kidney transplant, successfully treated with our protocol for focal and segmental glomerulosclerosis relapse. Recovery of CD19 cells counting was observed in six patients including two relapsing patients. No severe infection or neoplasia were reported. Conclusions: Rituximab successfully reduced relapsing in complicated idiopathic nephrotic syndrome, steroid dose and enabled other immunosuppressants weaning off in most patients. Recovery of CD19 cells did not anticipate relapse, but may herald the need for additional rituximab infusions.Centro Hospitalar do Porto2019-06-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articletext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003Nascer e Crescer v.28 n.2 2019reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003Moreira,Carla LealBaptista,RitaSousa,Pedro ManeiraMaciel,JulianaCosta,LianeTeixeira,AnaCosta,TeresaMatos,PaulaRocha,LilianaFaria,Maria SameiroMota,Conceiçãoinfo:eu-repo/semantics/openAccess2024-02-06T17:06:22Zoai:scielo:S0872-07542019000200003Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:19:46.079443Repositório 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 |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
title |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
spellingShingle |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience Moreira,Carla Leal nephrotic syndrome steroid-dependent steroid-resistant rituximab |
title_short |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
title_full |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
title_fullStr |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
title_full_unstemmed |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
title_sort |
Rituximab use in children with complicated idiopathic nephrotic syndrome - a single centre experience |
author |
Moreira,Carla Leal |
author_facet |
Moreira,Carla Leal Baptista,Rita Sousa,Pedro Maneira Maciel,Juliana Costa,Liane Teixeira,Ana Costa,Teresa Matos,Paula Rocha,Liliana Faria,Maria Sameiro Mota,Conceição |
author_role |
author |
author2 |
Baptista,Rita Sousa,Pedro Maneira Maciel,Juliana Costa,Liane Teixeira,Ana Costa,Teresa Matos,Paula Rocha,Liliana Faria,Maria Sameiro Mota,Conceição |
author2_role |
author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Moreira,Carla Leal Baptista,Rita Sousa,Pedro Maneira Maciel,Juliana Costa,Liane Teixeira,Ana Costa,Teresa Matos,Paula Rocha,Liliana Faria,Maria Sameiro Mota,Conceição |
dc.subject.por.fl_str_mv |
nephrotic syndrome steroid-dependent steroid-resistant rituximab |
topic |
nephrotic syndrome steroid-dependent steroid-resistant rituximab |
description |
Background: In children with idiopathic nephrotic syndrome, rituximab can maintain short-term remission allowing steroids and calcineurin inhibitors taper. Methods: We retrospectively reviewed all children receiving rituximab for idiopathic nephrotic syndrome at a tertiary hospital. All patients, except one who received a single dose, had four infusions of 375mg/m2 of rituximab at a one-week interval, minimum follow-up duration of four and maximum 118 months. Results: Eleven patients were included, three patients with steroid resistant nephrotic syndrome and eight steroid dependent or frequently relapsing nephrotic syndrome. Male:female ratio was 8:3 and median age at onset was 2.7 (minimum 1.7-maximum 9.9) years-old. All patients received a trial of calcineurin inhibitors, eight received cyclophosphamide and seven mycophenolate mofetil. Remission of proteinuria was observed in nine patients. Two patients were non-responders evolving to end-stage renal disease. Patients experienced in median three relapses during the six months preceding rituximab. Responders did not relapse in the following six months (p=0.013) and daily dose of prednisolone was significantly reduced (p=0.001). Two patients relapsed at 9 and 12 months after rituximab. Another patient experienced relapse immediately after kidney transplant, successfully treated with our protocol for focal and segmental glomerulosclerosis relapse. Recovery of CD19 cells counting was observed in six patients including two relapsing patients. No severe infection or neoplasia were reported. Conclusions: Rituximab successfully reduced relapsing in complicated idiopathic nephrotic syndrome, steroid dose and enabled other immunosuppressants weaning off in most patients. Recovery of CD19 cells did not anticipate relapse, but may herald the need for additional rituximab infusions. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-06-01 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003 |
url |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S0872-07542019000200003 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Centro Hospitalar do Porto |
publisher.none.fl_str_mv |
Centro Hospitalar do Porto |
dc.source.none.fl_str_mv |
Nascer e Crescer v.28 n.2 2019 reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799137287783579648 |