Hereditary angioedema and Allergic bronchopulmonary
aspergillosis: an unexpected association

Laise Fazanha Sgarbi; Sérgio Duarte Dortas-Junior; Maria Luiza Oliva Alonso; Alfeu Tavares França; Solange Oliveira Rodrigues Valle

doi:10.5935/2526-5393.20220015

Clinical and Experimental Communication

Hereditary angioedema and Allergic bronchopulmonary aspergillosis: an unexpected association

Angioedema hereditário e Aspergilose broncopulmonar alérgica: uma associação inesperada

Laise Fazanha Sgarbi; Sérgio Duarte Dortas-Junior; Maria Luiza Oliva Alonso; Alfeu Tavares França; Solange Oliveira Rodrigues Valle

http://dx.doi.org/10.5935/2526-5393.20220015 Arq Asma Alerg Imunol, vol.6, n1, p.141-143, 2022

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Abstract

Hereditary angioedema (HAE) is a rare autosomal dominant disorder, Allergic bronchopulmonary aspergillosis (ABPA) is a lung disease involving hypersensitivity to the fungi Aspergillus fumigatus which occur in susceptible patient with asthma or cystic fibrosis, also considered a rare disease. We report a case of HAE and ABPA in a single patient. HAE diagnosis was confirmed: C4 = 3 mg/dL, C1INH < 2.8 mg/dL - nephelometry. Former lung function showed elevation RV and RV/FVC, suggesting small airways lung disease. Positive skin prick test to Aspergillus fumigatus (03 mm); total serum IgE level 3,100 IU/mL (nephelometry - BNII Siemens), eosinophilia 11% (528/mm3) and specific A. fumigatus IgG antibodies 6,8 mgA/L (FEIA - fluorenzymeimmunoassay - ThermoFisher) and Chest CT showed mucoid impaction of the bronchi, consistent to current ABPA. Controlling ABPA could prevent and reduce angioedema attacks, and lung structural damage. Early diagnosis and treatment of both diseases should be emphasized to reduce mortality and morbidity

Keywords

Hereditary angioedema types I and II, allergic bronchopulmonary aspergillosis, asthma, bradykinin, hereditary angioedemas.

Resumo

Angioedema hereditário (AEH) é uma doença autossômica dominante; aspergilose broncopulmonar alérgica (ABPA) é uma doença de hipersensibilidade pulmonar relacionada ao esporo de Aspergillus fumigatus, mais suscetível em pacientes com asma e fibrose cística, ambas são consideradas doenças raras. Apresentamos um caso de AEH e ABPA em um paciente. O diagnóstico de AEH foi confirmado com exames laboratoriais: C4 = 3 mg/dL, C1INH < 2,8 mg/dL - nefelometria. Prova de função pulmonar evidenciou aumento de VR e VR/CVF, sugerindo doenças de pequenas vias aéreas. Teste de puntura positivo para A. fumigatus (03 mm); IgE total = 3.100 IU/mL (nefelometria - BNII Siemens), eosinofilia 11% (528/mm3) e IgG específica para A. fumigatus 6,8 mgA/L (FEIA - ThermoFisher), TC de tórax evidenciou impactação mucoide, consistente com ABPA. Controlar ABPA pode prevenir e reduzir as crises de angioedema e os danos ao tecido pulmonar. O diagnóstico precoce de ambas as doenças deve ser enfatizado para reduzir a morbimortalidade.

Palavras-chave

Angioedema hereditário tipos I e II, aspergilose broncopulmonar alérgica, asma, bradicinina, angioedemas hereditários.

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Submitted date:
11/21/2021

Accepted date:
12/01/2021