Bronquiectasias no relacionadas con fibrosis quística en niños: guías de diagnóstico, seguimiento y tratamiento / Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment

Las bronquiectasias se encuentran dentro del espectro de enfermedad pulmonar crónica caracterizada por la dilatación bronquial progresiva y, a menudo, irreversible, causada por cambios estructurales en la pared bronquial e inflamación crónica de las vías respiratorias. El síntoma cardinal es la tos crónica persistente húmeda y productiva, que debe alertar para realizar una intervención oportuna e interrumpir el ciclo de inflamación, infección y daño de la vía aérea. Un diagnóstico precoz a través de la tomografía axial computarizada de alta resolución de tórax y el monitoreo clínico facilitan la implementación de un tratamiento intensivo que reduce y minimiza el daño de la vía aérea. Si bien las acciones terapéuticas actuales para el manejo de bronquiectasias son efectivas, existen pocos estudios clínicos aleatorizados en pediatría. El objetivo del documento es proporcionar una actualización sobre el diagnóstico, seguimiento y tratamiento de las bronquiectasias no relacionadas con fibrosis quística en niños

Bronchiectasis is within the spectrum of chronic lung disease characterized by progressive and often irreversible bronchial dilation caused by structural changes in the bronchial wall and chronic inflammation of the airways. The cardinal symptom is persistent moist and productive chronic cough that should alert to timely intervention and interrupt the cycle of inflammation, infection, and airway damage. Early diagnosis through high-resolution computed tomography of the chest and clinical monitoring facilitate the implementation of intensive treatment that reduces and minimizes damage to the airway. Although current therapeutic actions for the management of bronchiectasis are effective, there are few randomized clinical trials in pediatrics. The objective of the document is to provide an update on the diagnosis, monitoring and treatment of bronchiectasis not related to cystic fibrosis in children

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.

Bronquiectasias: ¿no es más una enfermedad huérfana? / Bronchiectasis: is it no longer considered an orphan disease?

Bronchiectasis, so far considered an orphan disease, currently is diagnosed with a higher frequency due to several reasons such as renewed awareness of the disease, better diagnosis including imagenology, the development of patients registries, as well as a higher number of clinical research studies. The pathological basis of bronchiectasis is widely variable. Also the clinical expression is variable, from absence of symptoms in some patients up to chronic sputum production in others. Furthermore, a group of patients often develop recurrent exacerbations. Despite the etiologies of bronchiectasis are diverse, the main etiology is previous pulmonary infection. On the other hand, bronchiectasis could also be the expression of diverse systemic diseases. Even around one quarter of patients the etiology would not be established. The development of large registries of patients has allowed the building of classifications systems with accurate prognostic criteria. Chronic infection is the most relevant issue in bronchiectasis. Infection with P. aeruginosa has been associated with poor prognosis and their eradication must be attempted always. Effective secretions drainage techniques, oral and nebulized antibiotics, as well as mucolytic therapy are the mainstay of treatment in bronchiectasis.

Las bronquiectasias, consideradas hasta un tiempo atrás una enfermedad huérfana, se diagnostican actualmente con mayor frecuencia debido a un renovado interés en esta patología, a una mejoría de técnicas de diagnóstico, existencia de mejores registros, acceso a mejores imágenes y aumento de los estudios clínicos. El sustrato anátomo-patológico es notablemente variable, al igual que la expresión clínica, que va desde la ausencia de síntomas hasta la presencia de broncorrea crónica. A su vez, un grupo de pacientes tiende a presentar exacerbaciones frecuentes. Las etiologías de las bronquiectasias son múltiples, siendo la más frecuente la existencia de infecciones pulmonares previas. También pueden formar parte de enfermedades crónicas sistémicas. Sin embargo, en 25% de los casos no es posible identificar la etiología. El desarrollo de registros de pacientes ha permitido construir modelos de clasificación de gravedad, lo que hace posible establecer criterios pronósticos. La infección crónica es un hecho frecuente en bronquiectasias y la presencia de P aeruginosa confiere mal pronóstico a la enfermedad. La erradicación de Pseudomonas debe ser intentada siempre en estos pacientes. El uso de técnicas de drenaje, los antibióticos orales y nebulizados y las terapias mucolíticas constituyen los pilares centrales en el manejo de la enfermedad.

Alterações tomográficas pulmonares em mulheres não fumantes com DPOC por exposição à fumaça da combustão de lenha / Pulmonary changes on HRCT scans in nonsmoking females with COPD due to wood smoke exposure

OBJETIVO: Identificar e caracterizar alterações na TCAR de tórax em mulheres com DPOC causada por exposiçãoà fumaça da combustão de lenha. MÉTODOS: Foram selecionadas 42 pacientes com DPOC relacionada à exposição à fumaça de lenha, não fumantes, e 31 mulheres não fumantes e sem história de exposição à fumaça de lenha ou de doença pulmonar. Empregou-se um questionário para a obtenção de dados demográficos e informações sobre sintomas e exposições ambientais. Todas as participantes realizaram espirometria e TCAR de tórax. Os grupos DPOC e controle foram ajustados por idade, com 23 pacientes cada. RESULTADOS: A maioria das pacientes do grupo de estudo apresentava DPOC de leve a moderado (83,3%). Os achados de TCAR mais frequentes no grupo DPOC foram espessamento das paredes brônquicas, bronquiectasias, perfusão em mosaico, bandas parenquimatosas, padrão de árvore em brotamento e atelectasias laminares (p < 0,001 para todos na comparação com o grupo controle). As alterações, em geral, foram leves e de pequena extensão. Houve uma associação positiva entre espessamento das paredes brônquicas e duração da exposição à fumaça de lenha em horas-ano. O achado de enfisema centrolobular foi infrequente e não diferiu entre os grupos (p = 0,232). CONCLUSÕES: A exposição à fumaça de lenha provoca alterações predominantemente brônquicas, que podem ser detectadas por TCAR, mesmo nos casos de DPOC leve.

OBJECTIVE: To identify and characterize alterations seen on HRCT scans in nonsmoking females with COPD due to wood smoke exposure. METHODS: We evaluated 42 nonsmoking females diagnosed with wood smokerelated COPD and 31 nonsmoking controls with no history of wood smoke exposure or pulmonary disease. The participants completed a questionnaire regarding demographic data, symptoms, and environmental exposure. All of the participants underwent spirometry and HRCT of the chest. The COPD and control groups were adjusted for age (23 patients each). RESULTS: Most of the patients in the study group were diagnosed with mild to moderate COPD (83.3%). The most common findings on HRCT scans in the COPD group were bronchial wall thickening, bronchiectasis, mosaic perfusion pattern, parenchymal bands, tree-in-bud pattern, and laminar atelectasis (p < 0.001 vs. the control group for all). The alterations were generally mild and not extensive. There was a positive association between bronchial wall thickening and hour-years of wood smoke exposure. Centrilobular emphysema was uncommon, and its occurrence did not differ between the groups (p = 0.232). CONCLUSIONS: Wood smoke exposure causes predominantly bronchial changes, which can be detected by HRCT, even in patients with mild COPD.

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.

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.

Persistent asthma in adults: comparison of high resolution computed tomography of the lungs after one year of follow-up

OBJECTIVE: The aims of this study were to evaluate the role of high resolution computed tomography of the torax in detecting abnormalities in chronic asthmatic patients and to determine the behavior of these lesions after at least one year. METHOD: Fourteen persistent asthmatic patients with a mean forced expiratory volume in 1-second that was 63 percent of predicted and a mean forced expiratory volume in 1-second /forced vital capacity of 60 percent had two high resolution computed tomographys separated by an interval of at least one year. RESULTS: All 14 patients had abnormalities on both scans. The most common abnormality was bronchial wall thickening, which was present in all patients on both computed tomographys. Bronchiectasis was suggested on the first computed tomography in 5 of the 14 (36 percent) patients, but on follow-up, the bronchial dilatation had disappeared in 2 and diminished in a third. Only one patient had any emphysematous changes; a minimal persistent area of paraseptal emphysema was present on both scans. In 3 patients, a "mosaic" appearance was observed on the first scan, and this persisted on the follow-up computed tomography. Two patients had persistent areas of mucoid impaction. In a third patient, mucus plugging was detected only on the second computed tomography. CONCLUSIONS: We conclude that there are many abnormalities on the high resolution computed tomography of patients with persistent asthma. Changes suggestive of bronchiectasis, namely bronchial dilatation, frequently resolve spontaneously. Therefore, the diagnosis of bronchiectasis by high resolution computed tomography in asthmatic patients must be made with caution, since bronchial dilatation can be reversible or can represent false dilatation. Nonsmoking chronic asthmatic subjects in this study had no evidence of centrilobular or panacinar emphysema