Guidelines for the management and treatment of periodic fever syndromes
Autor(a) principal: | |
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Data de Publicação: | 2016 |
Outros Autores: | , , , , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFMG |
Texto Completo: | https://doi.org/10.1016/j.rbr.2015.08.007 http://hdl.handle.net/1843/40006 |
Resumo: | Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions. |
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2022-03-11T16:47:45Z2022-03-11T16:47:45Z20165614451https://doi.org/10.1016/j.rbr.2015.08.0072255-5021http://hdl.handle.net/1843/40006Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions.Objetivo: Estabelecer diretrizes baseadas em evidências científicas para manejo das Sín dromes periódicas associadas à criopirina (Criopirinopatias – CAPS). Descric¸ão do método de coleta de evidência: A Diretriz foi elaborada a partir de 4 questões clínicas que foram estruturadas por meio do P.I.C.O. (Paciente, Intervenc¸ão ou Indicador, Comparac¸ão e Outcome), com busca nas principais bases primárias de informac¸ão científica. Após definir os estudos potenciais para sustento das recomendac¸ões, estes foram graduados pela forc¸a da evidência e grau de recomendac¸ão. Resultado: Foram recuperados, e avaliados pelo título e resumo, 1215 artigos, tendo sido selecionados 42 trabalhos, para sustentar as recomendac¸ões. Recomendac¸ões: 1. O diagnóstico de CAPS é baseado na anamnese e manifestac¸ões clíni cas, sendo posteriormente confirmado por estudo genético. Pode se manifestar sob três fenótipos: FCAS (forma leve), MWS (forma intermediária) e CINCA (forma grave). Avaliac¸ões neurológica, oftalmológica, otorrinolaringológica e radiológica podem ser de grande valia na distinc¸ão entre as síndromes; 2. O diagnóstico genético com análise do gene NLRP3 deve ser conduzido nos casos suspeitos de CAPS, isto é, indivíduos que apresentam, antes dos 20 anos de idade, episódios recorrentes de inflamac¸ão expressa por urticária e febre moderada; 3. As alterac¸ões laboratoriais incluem leucocitose e elevac¸ão nos níveis séricos de proteínas inflamatórias; 4. Terapias alvo dirigidas contra a interleucina 1 levam a rápida remissão dos sintomas na maioria dos pacientes. Contudo, existem limitac¸ões importantes em relac¸ão à seguranc¸a em longo prazo. Nenhuma das três medicac¸ões anti-IL1 evita progressão das lesões ósseasengUniversidade Federal de Minas GeraisUFMGBrasilMEDICINA - FACULDADE DE MEDICINARevista brasileira de reumatologiaSíndrome autoinflamatóriaSíndrome neurológica cutânea e articular crônica infantilSíndrome de Muckle-WellsGuidelines for the management and treatment of periodic fever syndromesDiretrizes para o manejo e tratamento de síndromes febris periódicasinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articlehttps://www.scielo.br/j/rbr/a/gNVZtmsNQ8dJQdDBvd4b5kQ/abstract/?lang=enMaria Teresa R.a.terreriAna Júlia Pantoja de MoraesFlavio Roberto SztajnbokSheila Knupp Feitosa de OliveiraLucia Maria Arruda CamposMarcia BandeiraFlávia Patrícia Sena Teixeira SantosClaudia Saad MagalhãesWanderley Marques BernardoClaudio Arnaldo LenClovis Artur Almeida da SilvaCristina Medeiros Ribeiro de MagalhãesSilvana B. SacchettiVirgínia Paes Leme FerrianiDaniela Gerent Petry PiottoAndré de Souza Cavalcantiapplication/pdfinfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFMGinstname:Universidade Federal de Minas Gerais (UFMG)instacron:UFMGLICENSELicense.txtLicense.txttext/plain; charset=utf-82042https://repositorio.ufmg.br/bitstream/1843/40006/1/License.txtfa505098d172de0bc8864fc1287ffe22MD51ORIGINAL2016_Guidelines for the management and treatment of periodic fever syndromes.pdf2016_Guidelines for the management and treatment of periodic fever syndromes.pdfapplication/pdf771872https://repositorio.ufmg.br/bitstream/1843/40006/2/2016_Guidelines%20for%20the%20management%20and%20treatment%20of%20periodic%20fever%20syndromes.pdf98287e6415e7d85100770f0f1070148fMD521843/400062022-03-11 13:47:45.49oai:repositorio.ufmg.br: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Repositório de PublicaçõesPUBhttps://repositorio.ufmg.br/oaiopendoar:2022-03-11T16:47:45Repositório Institucional da UFMG - Universidade Federal de Minas Gerais (UFMG)false |
dc.title.pt_BR.fl_str_mv |
Guidelines for the management and treatment of periodic fever syndromes |
dc.title.alternative.pt_BR.fl_str_mv |
Diretrizes para o manejo e tratamento de síndromes febris periódicas |
title |
Guidelines for the management and treatment of periodic fever syndromes |
spellingShingle |
Guidelines for the management and treatment of periodic fever syndromes Maria Teresa R.a.terreri Síndrome autoinflamatória Síndrome neurológica cutânea e articular crônica infantil Síndrome de Muckle-Wells |
title_short |
Guidelines for the management and treatment of periodic fever syndromes |
title_full |
Guidelines for the management and treatment of periodic fever syndromes |
title_fullStr |
Guidelines for the management and treatment of periodic fever syndromes |
title_full_unstemmed |
Guidelines for the management and treatment of periodic fever syndromes |
title_sort |
Guidelines for the management and treatment of periodic fever syndromes |
author |
Maria Teresa R.a.terreri |
author_facet |
Maria Teresa R.a.terreri Ana Júlia Pantoja de Moraes Flavio Roberto Sztajnbok Sheila Knupp Feitosa de Oliveira Lucia Maria Arruda Campos Marcia Bandeira Flávia Patrícia Sena Teixeira Santos Claudia Saad Magalhães Wanderley Marques Bernardo Claudio Arnaldo Len Clovis Artur Almeida da Silva Cristina Medeiros Ribeiro de Magalhães Silvana B. Sacchetti Virgínia Paes Leme Ferriani Daniela Gerent Petry Piotto André de Souza Cavalcanti |
author_role |
author |
author2 |
Ana Júlia Pantoja de Moraes Flavio Roberto Sztajnbok Sheila Knupp Feitosa de Oliveira Lucia Maria Arruda Campos Marcia Bandeira Flávia Patrícia Sena Teixeira Santos Claudia Saad Magalhães Wanderley Marques Bernardo Claudio Arnaldo Len Clovis Artur Almeida da Silva Cristina Medeiros Ribeiro de Magalhães Silvana B. Sacchetti Virgínia Paes Leme Ferriani Daniela Gerent Petry Piotto André de Souza Cavalcanti |
author2_role |
author author author author author author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Maria Teresa R.a.terreri Ana Júlia Pantoja de Moraes Flavio Roberto Sztajnbok Sheila Knupp Feitosa de Oliveira Lucia Maria Arruda Campos Marcia Bandeira Flávia Patrícia Sena Teixeira Santos Claudia Saad Magalhães Wanderley Marques Bernardo Claudio Arnaldo Len Clovis Artur Almeida da Silva Cristina Medeiros Ribeiro de Magalhães Silvana B. Sacchetti Virgínia Paes Leme Ferriani Daniela Gerent Petry Piotto André de Souza Cavalcanti |
dc.subject.other.pt_BR.fl_str_mv |
Síndrome autoinflamatória Síndrome neurológica cutânea e articular crônica infantil Síndrome de Muckle-Wells |
topic |
Síndrome autoinflamatória Síndrome neurológica cutânea e articular crônica infantil Síndrome de Muckle-Wells |
description |
Objective: To establish guidelines based on cientific evidences for the management of cryopyrin associated periodic syndromes. Description of the evidence collection method: The Guideline was prepared from 4 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation. Results: 1215 articles were retrieved and evaluated by title and abstract; from these, 42 articles were selected to support the recommendations. Recommendations: 1. The diagnosis of CAPS is based on clinical history and clinical manifestations, and later confirmed by genetic study. CAPS may manifest itself in three phenotypes: FCAS (mild form), MWS (intermediate form) and CINCA (severe form). Neurological, ophthalmic, otorhinolaryngological and radiological assessments may be highly valuable in distinguishing between syndromes; 2. The genetic diagnosis with NLRP3 gene analysis must be conducted in suspected cases of CAPS, i.e., individuals presenting before 20 years of age, recurrent episodes of inflammation expressed by a mild fever and urticaria; 3. Laboratory abnormalities include leukocytosis and elevated serum levels of inflammatory proteins; and 4. Targeted therapies directed against interleukin-1 lead to rapid remission of symptoms in most patients. However, there are important limitations on the long-term safety. None of the three anti-IL-1β inhibitors prevents progression of bone lesions. |
publishDate |
2016 |
dc.date.issued.fl_str_mv |
2016 |
dc.date.accessioned.fl_str_mv |
2022-03-11T16:47:45Z |
dc.date.available.fl_str_mv |
2022-03-11T16:47:45Z |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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publishedVersion |
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http://hdl.handle.net/1843/40006 |
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https://doi.org/10.1016/j.rbr.2015.08.007 |
dc.identifier.issn.pt_BR.fl_str_mv |
2255-5021 |
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https://doi.org/10.1016/j.rbr.2015.08.007 http://hdl.handle.net/1843/40006 |
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2255-5021 |
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eng |
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eng |
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Revista brasileira de reumatologia |
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openAccess |
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Universidade Federal de Minas Gerais |
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Brasil |
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MEDICINA - FACULDADE DE MEDICINA |
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Universidade Federal de Minas Gerais |
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