Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review
Autor(a) principal: | |
---|---|
Data de Publicação: | 2023 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://doi.org/10.25753/BirthGrowthMJ.v32.i3.27759 |
Resumo: | Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs. |
id |
RCAP_ae074bcdcd9e27190d10bc76aee498a7 |
---|---|
oai_identifier_str |
oai:ojs.revistas.rcaap.pt:article/27759 |
network_acronym_str |
RCAP |
network_name_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository_id_str |
7160 |
spelling |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature ReviewVasculite de imunoglobulina A (púrpura de Henoch-Schönlein) em Pediatria - Revisão da LiteraturaReview ArticlesImmunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs.A vasculite de imunoglobulina A (VIgA) é uma vasculite de pequenos vasos que afeta sobretudo crianças. As principais manifestações clínicas são exantema purpúrico, proteinúria/hematúria, artralgia e dor abdominal. Este artigo apresenta uma revisão dos dados mais recentes de epidemiologia, diagnóstico e abordagem da VIgA em pediatria. A VIgA afeta 10 a 56 por 100.000 crianças/ano, manifestando-se inicialmente na idade média de 6 anos. Tanto fatores genéticos como ambientais contribuem para a sua patogénese, sendo que a deposição de imunocomplexos contendo imunoglobulina A1 anormalmente glicosilada desempenha um papel preponderante. Atendendo ao curso habitualmente benigno da doença, o tratamento conservador é geralmente suficiente. As complicações a curto prazo estão sobretudo relacionadas com envolvimento gastrointestinal, incluindo risco de perfuração e hemorragia. A morbilidade a longo prazo está associada a doença renal crónica e hipertensão. Os corticosteroides não estão recomendados na prevenção do envolvimento renal, mas podem ser úteis no tratamento, assim como outros fármacos imunossupressores.Centro Hospitalar Universitário de Santo António2023-11-16info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25753/BirthGrowthMJ.v32.i3.27759eng2183-9417Sousa, PatríciaCorreia de Oliveira, SusanaDias, ÂngelaTavares, Ana Cláudiainfo: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:RCAAP2023-11-16T17:28:52Zoai:ojs.revistas.rcaap.pt:article/27759Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T22:53:52.037977Repositório 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 |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review Vasculite de imunoglobulina A (púrpura de Henoch-Schönlein) em Pediatria - Revisão da Literatura |
title |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
spellingShingle |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review Sousa, Patrícia Review Articles |
title_short |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
title_full |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
title_fullStr |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
title_full_unstemmed |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
title_sort |
Immunoglobulin A vasculitis (Henoch-Schönlein purpura) in children - A Literature Review |
author |
Sousa, Patrícia |
author_facet |
Sousa, Patrícia Correia de Oliveira, Susana Dias, Ângela Tavares, Ana Cláudia |
author_role |
author |
author2 |
Correia de Oliveira, Susana Dias, Ângela Tavares, Ana Cláudia |
author2_role |
author author author |
dc.contributor.author.fl_str_mv |
Sousa, Patrícia Correia de Oliveira, Susana Dias, Ângela Tavares, Ana Cláudia |
dc.subject.por.fl_str_mv |
Review Articles |
topic |
Review Articles |
description |
Immunoglobulin A vasculitis (IgAV) is a small-vessel vasculitis that primarily affects children. Major manifestations include purpuric rash, proteinuria/hematuria, arthralgia, and abdominal pain. In this article, the authors review data on the epidemiology, diagnosis, and treatment of IgAV in children. IgAV affects 10 to 56 children per 100,000 per year. The mean age at presentation is six years. Both genetic and environmental factors contribute to its pathogenesis, but the deposition of immune complexes containing abnormal glycosylated immunoglobulin (Ig) A1 plays a predominant role. The course of the disease is usually benign and supportive care is sufficient. Short-term complications are mostly related to gastrointestinal involvement, including the risk of perforation and bleeding. Long-term morbidity is due to chronic kidney disease and hypertension. Corticosteroids are not recommended for prevention of renal involvement, but may be useful as a treatment strategy, as well as more aggressive immunosuppressive drugs. |
publishDate |
2023 |
dc.date.none.fl_str_mv |
2023-11-16 |
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 |
https://doi.org/10.25753/BirthGrowthMJ.v32.i3.27759 |
url |
https://doi.org/10.25753/BirthGrowthMJ.v32.i3.27759 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
2183-9417 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.publisher.none.fl_str_mv |
Centro Hospitalar Universitário de Santo António |
publisher.none.fl_str_mv |
Centro Hospitalar Universitário de Santo António |
dc.source.none.fl_str_mv |
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 |
repository.mail.fl_str_mv |
|
_version_ |
1799135136424394752 |