Arquivos de Asma, Alergia e Imunologia
https://aaai-asbai.org.br/article/doi/10.5935/2526-5393.20170005
Arquivos de Asma, Alergia e Imunologia
Artigo Especial

Diretrizes brasileiras para o diagnóstico e tratamento do angioedema hereditário – 2017

Brazilian guidelines for the diagnosis and treatment of hereditary angioedema – 2017

Pedro Giavina-Bianchi; L. Karla Arruda; Marcelo V. Aun; Regis A. Campos; Herberto J. Chong-Neto; Rosemeire N. Constantino-Silva; Fátima F. Fernandes; Maria F. Ferraro; Mariana P. L. Ferriani; Alfeu T. França; Gustavo Fusaro; Juliana F. B. Garcia; Shirley Komninakis; Luana S. M. Maia; Eli Mansour; Adriana S. Moreno; Antonio A. Motta; João Bosco Pesquero; Nathalia Portilho; Nelson A. Rosário; Faradiba S. Serpa; Dirceu Solé; Eliana Toledo; Solange O. R. Valle; Camila Lopes Veronez; Anete S. Grumach

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Resumo

O angioedema hereditário é uma doença autossômica dominante caracterizada por crises de edema com o envolvimento de múl‑ tiplos órgãos. A doença é desconhecida por muitos profissionais da área da saúde e, portanto, subdiagnosticada. Os pacientes que não são diagnosticados e tratados adequadamente têm uma mortalidade estimada de 25% a 40%, devido ao angioedema da laringe, resultando em asfixia. O angioedema de alças intesti‑ nais é outra manifestação importante e incapacitante, que pode ser a principal ou a única durante uma crise da doença. Neste cenário, um grupo de especialistas da Associação Brasileira de Alergia e Imunologia (ASBAI) e do Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH) atualizou as diretrizes para o diagnóstico e terapia do angioedema hereditário

Palavras-chave

Angioedema, angioedema hereditário, diagnóstico, tratamento.

Abstract

Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with the involvement of multiple organs. The disease is unknown to many health professionals and is therefore underdiagnosed. Patients who are not adequately diagnosed and treated have an estimated mortality rate ranging from 25% to 40%, due to laryngeal angioedema, which results in asphyxia. Angioedema affecting bowel loops is another important, incapacitating presentation that may be the main or only manifestation during a crisis. In this scenario, a group of experts affiliated with Associação Brasileira de Alergia e Imunologia (ASBAI) and Grupo de Estudos Brasileiro em Angioedema Hereditário (GEBRAEH) has updated the guidelines for the diagnosis and treatment of hereditary angioedema.

Keywords

Angioedema, hereditary angioedema, diagnosis, treatment.

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