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. 2019 Aug 12;45(4):e20190122.
doi: 10.1590/1806-3713/e20190122.

Brazilian consensus on non-cystic fibrosis bronchiectasis

[Article in English, Portuguese]
Mônica Corso Pereira  1 , Rodrigo Abensur Athanazio  2 , Paulo de Tarso Roth Dalcin  3   4 , Mara Rúbia Fernandes de Figueiredo  5 , Mauro Gomes  6   7 , Clarice Guimarães de Freitas  8 , Fernando Ludgren  9 , Ilma Aparecida Paschoal  1 , Samia Zahi Rached  2 , Rosemeri Maurici  10
Affiliations

Brazilian consensus on non-cystic fibrosis bronchiectasis

[Article in English, Portuguese]
Mônica Corso Pereira et al. J Bras Pneumol. .

Abstract
in English, Portuguese

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

Bronquiectasias têm se mostrado uma condição cada vez mais diagnosticada com a utilização da TCAR de tórax. Na literatura, a terminologia utilizada separa as bronquiectasias entre secundárias à fibrose cística e aquelas não associadas à fibrose cística, denominadas bronquiectasias não fibrocísticas neste documento. Muitas causas podem levar ao desenvolvimento de bronquiectasias, e o paciente geralmente tem sintomas crônicos de vias aéreas, infecções recorrentes e alterações tomográficas compatíveis com a condição. Em 2010, foi publicada a primeira diretriz internacional sobre diagnóstico e tratamento das bronquiectasias não fibrocísticas. No Brasil, este é o primeiro documento de revisão com o objetivo de sistematizar o conhecimento acumulado sobre o assunto até o momento. Como para vários tópicos do tratamento não há evidências suficientes para recomendações, optou-se aqui pela construção de um documento de consenso entre especialistas. A Comissão de Infecções Respiratórias da Sociedade Brasileira de Pneumologia e Tisiologia reuniu 10 pneumologistas com expertise em bronquiectasias no Brasil para avaliar criticamente as evidências científicas e diretrizes internacionais, assim como identificar aspectos relevantes à compreensão da heterogeneidade da doença bronquiectásica e a seu manejo diagnóstico e terapêutico. Foram determinados cinco grandes tópicos (fisiopatologia; diagnóstico; monitorização do paciente estável; tratamento do paciente estável; e manejo das exacerbações). Após essa subdivisão, os tópicos foram distribuídos entre os autores, que realizaram uma revisão não sistemática da literatura, priorizando as principais publicações nas áreas específicas, incluindo artigos originais e de revisão, assim como revisões sistemáticas. Os autores revisaram e opinaram sobre todos os tópicos, formando um documento único final que foi aprovado por todos.

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Figures

Figure 1
Figure 1. Pathophysiology of bronchiectasis: a “vicious cycle” of the various factors involved.
Figure 2
Figure 2. Chest HRCT scan. In A, tree-in-bud pattern (arrows). In B, mucoid impaction (mucus plug) in small airways (circles) and bronchial wall thickening (arrows).
Figure 3
Figure 3. Chest HRCT scan. Mosaic perfusion (or attenuation). Although present in A (inhalation), it is more visible in B (exhalation). The darker areas indicate air trapping due to small airways impairment, associated with oligemia.
Figure 4
Figure 4. Chest HRCT scan. In A, cystic bronchiectasis in fibroatelectatic areas. In B, varicose bronchiectasis (arrows) and cystic bronchiectasis (circle). Note the loss of bronchial generations (loss of millimetric branching) and impaired visualization of bronchovascular markings, suggestive of air trapping (even during inhalation).
Figure 5
Figure 5. Algorithm for the diagnosis and etiologic investigation of bronchiectasis. BMT: bone marrow transplantation; and Tx: transplantation.
Figure 6
Figure 6. Algorithm for the therapeutic management of stable bronchiectasis patients. BiPAP: bilevel positive airway pressure.
Figure 7
Figure 7. Flow chart for the therapeutic management of exacerbations. aIf intravenous treatment is necessary in non-severe exacerbations, consider the possibility of intravenous administration at home. bThe dose of ciprofloxacin, 750 mg, 12/12 h should be reserved for severe exacerbations in patients weighing more than 50 kg.
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