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.

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.

Síndrome de Lady Windermere como causa de tos crónica. A propósito de un caso / Lady Windemere syndrome as the cause of chronic cough. A propos of one case

Las micobacterias no tuberculosas son poco reconocidas hoy en el mundo, incrementándose su prevalencia a punto de partida de su sospecha en inmunodeprimidos o en enfermedades crónicas, como la fibrosis quística. Se presenta el caso de una paciente con diagnóstico de síndrome de Lady Windermere a punto de partida de un cuadro de tos crónica. Se realizó tomografía computarizada donde se evidenciaron bronquiectasias en el segmento lingular del pulmón izquierdo y en los lóbulos medio e inferior del derecho. Mediante la broncoscopia se aisló el complejo Mycobacterium avium-intracellulare. La falta de sospecha de micobacterias atípicas en pacientes sin factores predisponentes, con un cuadro de tos crónica, conduce al retraso en el diagnóstico y el deterioro clínico del paciente...

Nontuberculous mycobacteria are rarely recognized in the world today; their prevalence is increasing due to suspicion in immunocompromised patients or in those suffering from chronic illnesses such as cystic fibrosis. A case with of Lady Windermere Syndrome diagnosis that originally started with chronic cough is presented. Tomography showed bilateral bronchiectasis in the left lingual segment and in the middle and lower right lobe. Mycobacterium avium-intracellulare complex was isolated using bronchoscopy. The lack of suspicion of nontuberculous mycobacteria in patients without risk factors and chronic cough leads to diagnosis delay and clinical deterioration of the patient...

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.

Determinação do componente inflamatório das doenças das vias aéreas através do escarro induzido: utilização na prática clínica / Determination of the inflammatory component of airway diseases by induced sputum cell counts: use in clinical practice

OBJETIVO: Avaliar a utilização e a influência da determinação do componente inflamatório das doenças das vias aéreas (inflamometria), através do exame do escarro induzido, nas decisões terapêuticas de um serviço terciário de pneumologia. MÉTODOS: Foram analisadas 151 amostras de escarro induzido de 132 pacientes consecutivamente referidos para indução de escarro com propósitos clínicos por cinco pneumologistas, no período entre julho de 2006 e fevereiro de 2007. As indicações para a realização do teste e a conduta terapêutica adotada em função do resultado foram analisadas através de um questionário estruturado preenchido pelo médico que solicitou o escarro induzido. O escarro foi obtido e processado de acordo com uma técnica previamente descrita. RESULTADOS: As principais indicações do teste foram: titulação da dose do corticosteróide inalatório na asma moderada a grave (54,3%), investigação de tosse crônica (30,5%), monitoração da inflamação em pacientes com bronquiectasias (7,3 %) e monitoração da inflamação na doença pulmonar obstrutiva crônica (6%). Dos 82 pacientes com asma, 47 (57%) apresentaram bronquite eosinofílica (eosinófilos > 3%). Bronquite eosinofílica sem asma foi diagnosticada em 9 (19%) dos 46 pacientes que realizaram o exame para investigar tosse crônica. Bronquite neutrofílica (neutrófilos > 65%) foi observada em 13 pacientes; 5 com asma, 2 com tosse crônica e 6 com doença pulmonar obstrutiva crônica/bronquiectasias. Com base nos resultados do exame do escarro induzido, 48 (64,7%) pacientes com asma tiveram sua dose de corticosteróide modificada. CONCLUSÕES: A aplicação sistemática da inflamometria através do exame do escarro induzido pode trazer importantes benefícios aos pacientes com doenças respiratórias, principalmente àqueles portadores de asma e/ou tosse crônica.

OBJECTIVE: To evaluate the usefulness of determining the inflammatory component of airway diseases (inflammometry) by induced sputum cell counts, as well as its influence on treatment decisions in a tertiary facility for the treatment of respiratory diseases. METHODS: We analyzed 151 sputum samples from 132 consecutive patients referred for clinical sputum induction by five pulmonologists between July of 2006 and February of 2007. A structured questionnaire related to the reasons for requesting the test and to the therapeutic decision making based on test results was completed by each attending physician upon receiving the test results. Induced sputum was obtained and processed according to a technique previously described. RESULTS: The principal motives for ordering the test were inhaled corticosteroid dose titration in patients with moderate-to-severe asthma (in 54.3%), investigation of chronic cough (in 30.5%), and monitoring airway inflammation in patients with bronchiectasis (in 7.3%) or chronic obstructive pulmonary disease (in 6%). Of the 82 patients with asthma, 47 (57%) presented eosinophilic bronchitis (>3% eosinophils). Nonasthmatic eosinophilic bronchitis was diagnosed in 9 (19%) of the 46 patients with chronic cough. Neutrophilic bronchitis (>65% neutrophils) was found in 13 patients, of which 5 had asthma, 2 had chronic cough, and 6 had chronic obstructive pulmonary disease/bronchiectasis. Based on the induced sputum results, the corticosteroid dose was modified in 48 asthma patients (64.7%). CONCLUSIONS: The systematic application of inflammometry using induced sputum cell counts can be beneficial for patients with airway diseases, particularly those with asthma or chronic cough.

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.

Tomografia computadorizada de alta resuluçäo (TCAR) na avaliaçäo de alteraçöes nas pequenas vias aéreas de pacientes com supuraçäo brônquica crônica / High resolution CT scan in the evaluation of alterations in small airways of patients with chronic bronchial suppuration

As vias aéreas säo estrutura de dificil avaliaçäo por métodos de imagem. Desde a introduçäo da tomografia computadorizada de alta resoluçäo (TCAR), no início da década de 80, muitos avanços foram feitos na interpretaçäo dos sinais tomográficos de envolvimento das vias aérea, através de estudos de correlaçäo histológico-tomográficos. Este trabalho teve como objetivo estudar as anormalidade vista na TCAR nas pequenas r grandes vias aéreas, em um grupo de pacientes com quadro clínico de supuraçäo brônquica crônica, a fim de interpretar tais achados à luz do descrito na literatura. Em um grupo de 36 pacientes que procuraram os ambulatórios de Pneumologia do Hospital de Clínica da Unicamp com quadro de supuraçäo brônquica crônica, foi realizada avaliaçäo clínica-laboratorial rotineira para bronquiectasia e exame de tomografia computadorizada de alta resoluçäo. Dentre os 36 doentes foram encontrados 12 portadores de fibrose cística e 24 com outros diagnósticos, mencionados no texto. Os achados tomográficos säo descritos e classificados na tentativa de facilitar o reconhecimento e a interpretaçäo dessas alteraçöes. A TCAR permite a visibilizaçäo das pequenas e grandes vias aéreas com precisäo e, a patir de dados de literatura sobre a correlaçäo histológica-tomográfica, podem-se inferir dados sobre a fisiopatologenia da doença