Bronquiectasias no fibrosis quística: desde la infancia a la adultez: enfoque diagnostico y terapéutico / Bronchiectasis no cystic fibrosis: from childhood to adult: diagnostic and therapeutic approach

Bronchiectasis is a suppurative lung disease with heterogeneous phenotypic characteristics. It is defined as abnormal dilation of the bronchi, losing the existing relationship between bronchial sizes and accompanying artery. According to their form, they can be cylindrical, varicose, saccular or cystic. According to its location, they could be diffuse or localized. The diagnosis of bronchiectasis is usually suspected in patients with chronic cough, mucopurulent bronchorrea, and recurrent respiratory infections. The etiology can be varied, being able to classify in cystic fibrosis bronchiectasis, when there is cystic fibrosis transmembrane regulator (CFTR) gene mutation and not cystic fibrosis, being post infectious the most frequent. Its relationship with childhood is unknown. Severe respiratory infections can predispose in a susceptible subject the so-called theory of the "vicious circle" and the development of these. Persistent bacterial bronchitis in children has been described as a probable cause of not cystic fibrosis bronchiectasis in adults. The treatment is based on the management of symptoms and the prevention of exacerbations. The evidence is poor and many treatments are extrapolated from cystic fibrosis bronchiectasis. We are going to describe the diagnostic and therapeutic approach of non-cystic fibrosis bronchiectasis in adults.

La bronquiectasia es una enfermedad pulmonar supurativa con características fenotípicas heterogéneas. Se define como la dilatación anormal de los bronquios, perdiendo la relación existente entre tamaño bronquial y arteria que acompaña. Según su forma, pueden ser clasificadas en cilíndricas, varicosas, saculares o quísticas y según su etiología presentarse de forma difusa o localizada. El diagnóstico de bronquiectasias se sospecha generalmente en pacientes con tos crónica, broncorrea mucosa, mucupurulenta e infecciones respiratorias recurrentes. La etiología es variada, pudiendo clasificarse en bronquiectasias fibrosis quística, aquellas que se encuentran en el contexto de la mutación del gen regulador transmembrana de fibrosis quística (CFTR) y no fibrosis quística, de etiologías diversas, siendo post infecciosas la gran mayoría. No se conoce con certeza su relación con la infancia, es sabido que infecciones respiratorias severas pueden predisponer en un sujeto susceptible, a la llamada teoría del "circulo vicioso" y el desarrollo de estas. La bronquitis bacteriana persistente en niños se ha descrito como una causa probable del desarrollo de bronquiectasias no fibrosis quística en adultos. El tratamiento se basa en el manejo de los síntomas y la prevención de las exacerbaciones. La evidencia es escasa y la mayoría de las terapias se han investigado en las bronquiectasias tipo fibrosis quística. En este trabajo se explicará el enfrentamiento diagnóstico y terapéutico de los adultos portadores de bronquiectasias no fibrosis quística.

Brazilian consensus on non-cystic fibrosis bronchiectasis / Consenso brasileiro sobre bronquiectasias não fibrocísticas

ABSTRACT 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.

RESUMO 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.

Infección crónica por Pseudomonas aeruginosa en pacientes con bronquiectasias no fibrosis quística / Chronic infection by Pseudomonas aeruginosa in patients with non-cystic fibrosis bronchiectasias

Resumen Introducción: La infección crónica por Pseudomonas aeruginosa (PA) es frecuente en pacientes con bronquiectasias (BQ) y representa un quiebre en la historia natural de la enfermedad, asociándose a mal pronóstico y mayor severidad. Objetivo: Caracterizar la población portadora de BQ no fibrosis quística (BQ no-FQ) del Instituto Nacional del Tórax (INT) infectados crónicamente con PA comparándolos con pacientes que mantienen cultivos de expectoración negativos para este germen. Metodología: Revisión retrospectiva de fichas clínicas de pacientes portadores de BQ del INT entre julio de 2007 y abril de 2017. Se caracterizó la población y se comparó score de FACED y otros índices de gravedad. Resultados: El promedio de edad fue de 55 ± 17,3 años, 81% de los pacientes fue de género femenino. De acuerdo a aislamiento de Pseudomonas en cultivo esputo se clasificaron como infectados crónicamente (BQ con PA; n = 61) y no infectados con PA (BQ sin PA; n = 59). No hubo diferencias entre los grupos en edad y sexo. El VEF1 fue más bajo en el grupo con PA los que tienen más hospitalizaciones. Se calculó el índice de riesgo FACED siendo mayor en los pacientes colonizados. La etiología más frecuente es la postinfecciosa, principalmente secuelas de TBC, con 30,8% de etiología no identificada. Conclusiones: Los pacientes con bronquiectasias con infección crónica por Pseudomonas aeruginosa tienen una enfermedad más severa, con VEF1 más bajo, y con mayor índice de severidad de FACED. Destaca en nuestro grupo la etiología postinfecciosa.

Introduction: Chronic airways infection with Pseudomonas aeruginosa (PA) is a common situation in patients with Bronchiectasis (BQ) and constitutes a breakdown in the natural history of the latter. Moreover, BQ is also associated with a poor prognosis and an increased severity of the disease. Objective: To describe the characteristics of the population diagnosed with non-Cystic Fibrosis Bronchiectasis (non-CFB) who are chronically infected with PA, and to perform a comparison with patients with negative sputum cultures. Methodology: We performed a retrospective analysis of the clinical files of patients diagnosed with non-CFB who were attended at the 'Instituto Nacional del Tórax' (Chile) between July 2007 and April 2017. The characteristics of the population were described and the FACED scores and other severity indexes were compared. Results: The average age of patients was 55 ± 17.3 years-old, and 81% of them were female. According to PA isolation in sputum culture, they were classified as "chronically infected" (non-CFB with PA, n = 61) and "not infected with PA" (non-CFB without PA, n = 59). There were no differences in age and gender between the two groups. On the other hand, FEV1 was lower in the non-CFB PA group. The calculated FACED score was higher in colonized patients. The most frequent etiology was post-infectious, mainly TB sequels, with a 30.8% unidentified etiology. Conclusions: Patients with bronchiectasis chronically infected with Pseudomonas aeruginosa show increases in the severity of the disease, with a lower FEV1 and a higher FACED score. The postinfectious etiology is highlighted in our group.

Airway disease: similarities and differences between asthma, COPD and bronchiectasis

Airway diseases are highly prevalent worldwide; however, the prevalence of these diseases is underestimated. Although these diseases present several common characteristics, they have different clinical outcomes. The differentiation between asthma, chronic obstructive pulmonary disease and bronchiectasis in the early stage of disease is extremely important for the adoption of appropriate therapeutic measures. However, because of the high prevalence of these diseases and the common pathophysiological pathways, some patients with different diseases may present with similar symptoms. The objective of this review is to highlight the similarities and differences between these diseases in terms of the risk factors, pathophysiology, symptoms, diagnosis and treatment.

Distância percorrida no teste de caminhada de seis minutos não se relaciona com qualidade de vida em pacientes com bronquiectasias não fibrocísticas / Six-minute walk distance is not related to quality of life in patients with non-cystic fibrosis bronchiectasis

OBJETIVO: Avaliar o desempenho físico de pacientes com bronquiectasias não fibrocísticas no teste de caminhada de seis minutos (TC6) e investigar sua associação com a qualidade de vida (QV). Secundariamente, analisar a associação entre a distância percorrida no TC6 (DTC6) com achados clínicos e espirométricos para se identificar preditores para esse desempenho. MÉTODOS: Estudo transversal envolvendo pacientes com bronquiectasias não fibrocísticas, com idade > 18 anos, pelo menos um sintoma respiratório por > 2 anos e VEF1 < 70% do previsto. Os pacientes foram submetidos a avaliação clínica, teste de função pulmonar, TC6 e avaliação da QV por Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). RESULTADOS: Foram incluídos 70 pacientes (48 mulheres; média de idade = 54,5 ± 17,7 anos; média de VEF1 = 44,9 ± 14,5% do previsto. Os pacientes foram divididos em dois grupos: DTC6-menor, com desempenho menor que o limite inferior previsto (n = 23); e DTC6-norm, com desempenho normal (n = 47). Em comparação ao grupo DTC6-norm, o grupo DTC6-menor apresentou menor idade, menor idade ao diagnóstico das bronquiectasias, menor proporção de ex-fumantes, menor índice de massa corpórea (IMC), menor VEF1 em % do previsto e menor PEmáx em % do previsto. Não houve diferenças significativas nos escores do SF-36 entre os grupos. No modelo de regressão logística, menor idade e menor IMC se associaram significativamente com menor DTC6. CONCLUSÕES: Nesta amostra, uma elevada proporção de pacientes apresentou uma DTC6 menor que o esperado. A DTC6 não se relacionou com a QV. Idade e IMC se associaram a DTC6.

OBJECTIVE: To evaluate physical performance on the six-minute walk test (6MWT) in patients with non-cystic fibrosis bronchiectasis and to investigate its relationship with quality of life (QoL). To identify predictors of exercise performance, we also investigated whether six-minute walk distance (6MWD) is associated with clinical and spirometric findings. METHODS: This was a cross-sectional study involving patients with non-cystic fibrosis bronchiectasis (age, > 18 years), with at least one respiratory symptom for > 2 years and an FEV1 < 70% of predicted. Patients underwent clinical evaluation, pulmonary function tests, the 6MWT, and QoL assessment with the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36). RESULTS: We included 70 patients (48 females). Mean age was 54.5 ± 17.7 years, and mean FEV1 was 44.9 ± 14.5% of predicted. The patients were divided into two groups: 6MWD-low (6MWD below the predicted lower limit; n = 23); and 6MWD-norm (normal 6MWD; n = 47). The following variables were significantly lower in the 6MWD-low group than in the 6MWD-norm group: age; age at diagnosis of bronchiectasis; proportion of former smokers; body mass index (BMI); FEV1% of predicted; and MEP% of predicted. There were no significant differences in the SF-36 scores between the groups. In the logistic regression model, lower age and lower BMI were significantly associated with lower 6MWD. CONCLUSIONS: In this sample, there was a high proportion of patients who presented a lower than expected 6MWD. Although 6MWD was not related to QoL, it was associated with age and BMI.

Bronquiectasias en adultos: características clínicas: experiencia de 5 años 1998-2003 / Retrospective evaluation of bronchiectasis characteristics in adults at a chilean general hospital: a 5 years experience (1998-2003)

In order to know the actual characteristics of bronchiectasis in an adult population, we reviewed 18 cases with this pathology confirmed by spiral CT at a general hospital (Hospital Regional de Concepción, Chile), between 1998-2003. Ten patients were males and 8 females, their mean age was 44 +/- 13.9 years old. Most common etiologies of bronchiectasis were pulmonary tuberculosis (44.4 percent) and acute pneumonia (38.8 percent); main sypmtoms were chronic cough (88.8 percent), persistent sputum (77.7 percent) and hemoptysis (44.4 percent). Spirometry detected airway obstruction in smokers (FEV1/FVC = 58 percent) and in non smokers (FEV1/FVC = 68 percent). Microbiological sputum exams showed Hemophilus influenzae (16.6 percent), Neisseria catharralis (15 percent), Pseudomona aeruginosa (13 percent), Streptococcus pneumoniae (15.4 percent) and Candida albicans (4 percent). Spiral CT showed the following distribution of bronchiectasis: unilobular lesion 11.1 percent, bilobar 72 percent and multilobar 16.6 percent. The clinical picture of bronchiectasis in our hospital according to this up to date review, shows that our reality is essentially similar to that previously reported in literature.

Para conocer las características actuales de las bronquiectasias en adultos en nuestro medio se revisan los 18 casos de esta patología comprobados por TAC helicoidal de tórax en el Hospital Regional de Concepción entre 1998-2003. La edad promedio de los pacientes fue de 44 +/ - 13,9 años; 10 hombres y 8 mujeres. Las etiologías más frecuentes de bronquiectasias fueron tuberculosis pulmonar (44,4 por ciento) y neumonía aguda (38,8 por ciento). Los síntomas más relevantes fueron tos crónica (88,8 por ciento), expectoración persistente (77,7 por ciento) y hemoptisis (44,8 por ciento). La espirometría reveló alteración ventilatoria obstructiva tanto en fumadores (VEF1/CVF = 58 por ciento) como en no fumadores (VEF1/CVF = 68 por ciento). La bacteriología de expectoración detectó Hemophilus influenzae (16,6 por ciento), Neisseria catharralis (15 por ciento), Pseudomona aeruginosa (13 por ciento), Streptococcus pneumoniae (15,4 por ciento) y Candida albicans (4 por ciento). La TAC de tórax demostró lesiones bilobares (72 por ciento), multilobares (16,6 por ciento) y unilobares (11,1 por ciento). La realidad de las bronquiectasias en nuestro medio es que no difiere significativamente de lo descrito en la literatura.