Neonatal lupus erythematosus: A narrative review
Autor(a) principal: | |
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Data de Publicação: | 2022 |
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.v31.i4.27109 |
Resumo: | Introduction: Neonatal lupus erythematosus (NLE) is characterized by the transplacental passage of maternal antibodies, mainly anti-Sjogren’s syndrome A/Ro (anti-SSA/Ro) and anti-Sjogren’s syndrome B/La (anti-SSB/La), which bind to the developing fetal tissues. Seropositive mothers may have a diagnosed disease, such as Sjögren’s syndrome, systemic lupus erythematosus, or undifferentiated connective tissue disease, but in 25─60% of cases, they are asymptomatic at the time of childbirth. Objectives: This article aimed to provide a narrative review of the current state of knowledge on NLE. Development: The clinical spectrum of NLE includes both reversible and irreversible features. Reversible clinical features, like cutaneous lesions, hematological and pulmonary abnormalities, and hepatobiliary dysfunction, disappear spontaneously as autoantibody levels decrease in the bloodstream. The most commonly reported NLE manifestation is cardiac disease. Injury to the cardiac conduction tissue may lead to defects, such as congenital atrioventricular (AV) block, which is the most severe and, in some cases, lethal NLE manifestation. NLE may also have an impact on the myocardium and endocardium, with maternal antibodies being responsible for endocardial fibroelastosis. Immune-mediated AV block may benefit from in-utero treatment with steroids. Hydroxychloroquine may also be prescribed to women with immune-mediated disorders in the preconception period. Conclusions: The management of infants affected with NLE should be performed in a tertiary care center. Postnatal pacemaker insertion may be indicated in the presence of irreversible AV block or heart failure. Erythrocyte or platelet transfusions, corticosteroids, or intravenous immunoglobulin may be indicated to treat severe disease manifestations. |
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Neonatal lupus erythematosus: A narrative reviewLupus eritematoso neonatal: Uma revisão narrativaReview ArticlesIntroduction: Neonatal lupus erythematosus (NLE) is characterized by the transplacental passage of maternal antibodies, mainly anti-Sjogren’s syndrome A/Ro (anti-SSA/Ro) and anti-Sjogren’s syndrome B/La (anti-SSB/La), which bind to the developing fetal tissues. Seropositive mothers may have a diagnosed disease, such as Sjögren’s syndrome, systemic lupus erythematosus, or undifferentiated connective tissue disease, but in 25─60% of cases, they are asymptomatic at the time of childbirth. Objectives: This article aimed to provide a narrative review of the current state of knowledge on NLE. Development: The clinical spectrum of NLE includes both reversible and irreversible features. Reversible clinical features, like cutaneous lesions, hematological and pulmonary abnormalities, and hepatobiliary dysfunction, disappear spontaneously as autoantibody levels decrease in the bloodstream. The most commonly reported NLE manifestation is cardiac disease. Injury to the cardiac conduction tissue may lead to defects, such as congenital atrioventricular (AV) block, which is the most severe and, in some cases, lethal NLE manifestation. NLE may also have an impact on the myocardium and endocardium, with maternal antibodies being responsible for endocardial fibroelastosis. Immune-mediated AV block may benefit from in-utero treatment with steroids. Hydroxychloroquine may also be prescribed to women with immune-mediated disorders in the preconception period. Conclusions: The management of infants affected with NLE should be performed in a tertiary care center. Postnatal pacemaker insertion may be indicated in the presence of irreversible AV block or heart failure. Erythrocyte or platelet transfusions, corticosteroids, or intravenous immunoglobulin may be indicated to treat severe disease manifestations.Introdução: O lúpus eritematoso neonatal caracteriza-se pela passagem transplacentária de anticorpos maternos, maioritariamente anti-síndrome de Sjögren A/Ro (anti-SSA/Ro) e anti-síndrome de Sjögren B/La (anti-SSB/La), que se ligam aos tecidos fetais em desenvolvimento. Mães seropositivas podem ter doença diagnosticada, nomeadamente síndrome de Sjögren, lúpus eritematoso sistémico, ou doença indiferenciada do tecido conjuntivo, mas em 25─60% dos casos são assintomáticas à data do parto. Objetivos: O objetivo deste estudo foi fazer uma revisão narrativa sobre o estado da arte do lúpus eritematoso neonatal. Desenvolvimento: As características clínicas do lúpus neonatal podem ser reversíveis ou irreversíveis. As características reversíveis, como lesões cutâneas, alterações hematológicas ou pulmonares e disfunção hepatobiliar, desaparecem espontaneamente à medida que os níveis de anticorpos circulantes diminuem, geralmente nos primeiros 6 a 8 meses de vida. A manifestação mais frequente de lúpus neonatal é doença cardíaca. A lesão do tecido de condução cardíaco pode resultar em bloqueio atrioventricular (AV) congénito, que constitui a manifestação mais grave e em alguns casos fatal de lúpus neonatal. A doença pode também ter impacto no miocárdio e endocárdio, dado que os anticorpos maternos podem causar fibroelastose endocárdica. O bloqueio AV imunomediado pode beneficiar do tratamento in utero com corticoides. Mulheres com patologia imunomediada podem também receber hidroxicloroquina no período pré-concecional. Conclusões: A abordagem de recém-nascidos com lúpus neonatal deve ser feita num centro terciário. Em presença de bloqueio AV irreversível ou sinais de insuficiência cardíaca, pode ser necessária a colocação de pacemaker após o nascimento. As manifestações graves da doença podem necessitar de transfusões de eritrócitos ou plaquetas, corticosteroides ou imunoglobulina endovenosa.Centro Hospitalar Universitário do Porto2022-12-27info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://doi.org/10.25753/BirthGrowthMJ.v31.i4.27109eng2183-9417Marques, Ana RaquelRocha, GustavoSoares, PauloRodrigues, MarianaPimenta, Joanainfo: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:RCAAP2022-12-29T17:00:49Zoai:ojs.revistas.rcaap.pt:article/27109Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:29:01.993492Repositório 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 |
Neonatal lupus erythematosus: A narrative review Lupus eritematoso neonatal: Uma revisão narrativa |
title |
Neonatal lupus erythematosus: A narrative review |
spellingShingle |
Neonatal lupus erythematosus: A narrative review Marques, Ana Raquel Review Articles |
title_short |
Neonatal lupus erythematosus: A narrative review |
title_full |
Neonatal lupus erythematosus: A narrative review |
title_fullStr |
Neonatal lupus erythematosus: A narrative review |
title_full_unstemmed |
Neonatal lupus erythematosus: A narrative review |
title_sort |
Neonatal lupus erythematosus: A narrative review |
author |
Marques, Ana Raquel |
author_facet |
Marques, Ana Raquel Rocha, Gustavo Soares, Paulo Rodrigues, Mariana Pimenta, Joana |
author_role |
author |
author2 |
Rocha, Gustavo Soares, Paulo Rodrigues, Mariana Pimenta, Joana |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Marques, Ana Raquel Rocha, Gustavo Soares, Paulo Rodrigues, Mariana Pimenta, Joana |
dc.subject.por.fl_str_mv |
Review Articles |
topic |
Review Articles |
description |
Introduction: Neonatal lupus erythematosus (NLE) is characterized by the transplacental passage of maternal antibodies, mainly anti-Sjogren’s syndrome A/Ro (anti-SSA/Ro) and anti-Sjogren’s syndrome B/La (anti-SSB/La), which bind to the developing fetal tissues. Seropositive mothers may have a diagnosed disease, such as Sjögren’s syndrome, systemic lupus erythematosus, or undifferentiated connective tissue disease, but in 25─60% of cases, they are asymptomatic at the time of childbirth. Objectives: This article aimed to provide a narrative review of the current state of knowledge on NLE. Development: The clinical spectrum of NLE includes both reversible and irreversible features. Reversible clinical features, like cutaneous lesions, hematological and pulmonary abnormalities, and hepatobiliary dysfunction, disappear spontaneously as autoantibody levels decrease in the bloodstream. The most commonly reported NLE manifestation is cardiac disease. Injury to the cardiac conduction tissue may lead to defects, such as congenital atrioventricular (AV) block, which is the most severe and, in some cases, lethal NLE manifestation. NLE may also have an impact on the myocardium and endocardium, with maternal antibodies being responsible for endocardial fibroelastosis. Immune-mediated AV block may benefit from in-utero treatment with steroids. Hydroxychloroquine may also be prescribed to women with immune-mediated disorders in the preconception period. Conclusions: The management of infants affected with NLE should be performed in a tertiary care center. Postnatal pacemaker insertion may be indicated in the presence of irreversible AV block or heart failure. Erythrocyte or platelet transfusions, corticosteroids, or intravenous immunoglobulin may be indicated to treat severe disease manifestations. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-12-27 |
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.v31.i4.27109 |
url |
https://doi.org/10.25753/BirthGrowthMJ.v31.i4.27109 |
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 do Porto |
publisher.none.fl_str_mv |
Centro Hospitalar Universitário do Porto |
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 |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799130748226109440 |