Angioedema hereditário e outras formas de angioedema por bradicinina: atualização no diagnóstico e tratamento
Hereditary angioedema and other forms of bradykinin-mediated angioedema: update on diagnosis and treatment
Maria Fernanda Ferraro; L. Karla Arruda; Luana S. M. Maia; Adriana S. Moreno
Resumo
Angioedema é definido como edema que ocorre em áreas bem delimitadas do tecido subcutâneo e submucoso, consequente ao aumento da permeabilidade capilar local causada por mediadores vasoativos. Em geral acomete extremidades, face, vias aéreas superiores e tratos gastrointestinal e geniturinário. Há dois mediadores amplamente reconhecidos na patogênese do angioedema: histamina e bradicinina, com repercussões clínicas distintas. O angioedema histaminérgico é geralmente associado a urticária e tem boa resposta ao tratamento com anti-histamínicos, corticosteroides e adrenalina. Já o angioedema por bradicinina tem duração mais prolongada (36 a 72 horas), envolve mais frequentemente o trato gastrointestinal e não responde ao tratamento convencional com anti-histamínicos, corticosteroides e adrenalina. Suspeita-se de angioedema mediado por bradicinina quando há angioedema recorrente, não associado a urticária, que pode ser hereditário ou adquirido. O nonapeptídeo bradicinina é um potente mediador de vasodilatação e aumento da permeabilidade vascular, particularmente em vênulas pós-capilares, que produz seus efeitos através da estimulação dos receptores B2 ligados a proteína-G. Neste artigo de revisão, temos por objetivo fazer uma atualização em diagnóstico diferencial e tratamento das diferentes formas de angioedema mediado por bradicinina: angioedema por inibidores da enzima conversora da angiotensina (iECA) e outros fármacos que podem diminuir o metabolismo da bradicinina; angioedema hereditário (AEH) por mutações nos genes SERPING1 e F12 que codificam, respectivamente, o inibidor de C1-INH e o fator XII da coagulação; angioedema hereditário de causa desconhecida; angioedema por deficiência adquirida do C1-INH; e angioedema idiopático.
Palavras-chave
Abstract
Angioedema is defined as edema that occurs in well-defined areas of subcutaneous and submucosal tissues, due to increase in local capillary permeability caused by vasoactive mediators. In general, the edema takes place in extremities, face, upper airways and gastrointestinal and genitourinary tracts. Two main mediators have been widely recognized in the pathogenesis of angioedema: histamine and bradykinin, with distinct clinical implications. Histaminergic angioedema is often associated with urticaria and presents good response to antihistamines, corticosteroids and epinephrine. On the other hand, bradykinin-mediated angioedema lasts longer (36 to 72 hours), involves the gastrointestinal tract often, and does not respond to conventional treatment with antihistamines, corticosteroids and epinephrine. The presence of recurrent episodes of angioedema, not associated with urticaria, raises the suspicion of bradykinin-induced angioedema, which can be hereditary or acquired. The nonapeptide bradykinin is a potent mediator of vasodilation and increase in vascular permeability, particularly in post capillary venules, which produces its effects through stimulation of protein G-coupled B2 receptors. In this review article, we have aimed to carry out an update in the differential diagnosis and treatment of the distinct forms of bradykinin-mediated angioedema: angioedema induced by angiotensin-converting enzyme inhibitors (ACEi) and other drugs which could decrease metabolism of bradykinin; hereditary angioedema (HAE) due to mutations in the genes SERPING1 and F12, which encode C1-inhibitor (C1-INH) and coagulation factor XII, respectively; hereditary angioedema of unknown cause; angioedema due to acquired deficiency of C1-INH; and idiopathic angioedema.
Keywords
Referências
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Submetido em:
12/05/2015
Aceito em:
16/05/2015
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