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.

Association of body mass index with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis

The objective of this observational, multicenter study was to evaluate the association of body mass index (BMI) with disease severity and prognosis in patients with non-cystic fibrosis bronchiectasis. A total of 339 patients (197 females, 142 males) diagnosed with non-cystic fibrosis bronchiectasis by high-resolution computed tomography were classified into four groups: underweight (BMI<18.5 kg/m2), normal weight (18.5≤BMI<25.0 kg/m2), overweight (25.0≤BMI<30.0 kg/m2), and obese (BMI≥30.0 kg/m2). Clinical variables expressing disease severity were recorded, and acute exacerbations, hospitalizations, and survival rates were estimated during the follow-up period. The mean BMI was 21.90 kg/m2. The underweight group comprised 28.61% of all patients. BMI was negatively correlated with acute exacerbations, C-reactive protein, erythrocyte sedimentation rate, radiographic extent of bronchiectasis, and chronic colonization by P. aeruginosa and positively correlated with pulmonary function indices. BMI was a significant predictor of hospitalization risk independent of relevant covariates. The 1-, 2-, 3-, and 4-year cumulative survival rates were 94%, 86%, 81%, and 73%, respectively. Survival rates decreased with decreasing BMI (χ2=35.16, P<0.001). The arterial carbon dioxide partial pressure, inspiratory capacity, age, BMI, and predicted percentage of forced expiratory volume in 1 s independently predicted survival in the Cox proportional hazard model. In conclusion, an underweight status was highly prevalent among patients with non-cystic fibrosis bronchiectasis. Patients with a lower BMI were prone to developing more acute exacerbations, worse pulmonary function, amplified systemic inflammation, and chronic colonization by P. aeruginosa. BMI was a major determinant of hospitalization and death risks. BMI should be considered in the routine assessment of patients with non-cystic fibrosis bronchiectasis.

Reproducibility of step tests in patients with bronchiectasis / Reprodutibilidade de testes do degrau em pacientes bronquiectasicos

BACKGROUND: The step test has been used to assess exercise capacity in patients with chronic respiratory disease; however, its use has not been described with regard to patients with bronchiectasis (BCT). OBJECTIVE: This study assessed the reliability of the Chester step test (CST) and the modified incremental step test (MIST) and also correlated these tests with pulmonary function, heart rate (HR), and distance walked during the 6-min walk test (6-MWT). METHOD: On separate days, 17 patients randomly underwent two CSTs, two MISTs, and two 6-MWTs. Number of steps (NOSs), HR, and perceived exertion were recorded immediately before and after these tests. RESULTS: NOSs were similar across CSTs (124±65 and 125±67) and MISTs (158±83 and 156±76). Differences were not found across the CSTs and MISTs with regard to HR (138±25 bpm and 136±27 bpm), SpO2 (91±5% and 91±3%), perceived exertion (dyspnea=4 [3-5] and 4 [2-4.5]) and fatigue (4 [2-6] and 4 [3-5]). The CST was significantly briefer than the MIST (6.0±2.2 min and 8.6±3.0 min) and had fewer associated NOS (125±67 and 158±83). NOSs were correlated with FEV1, the 6-MWD, and HR for both tests. CONCLUSIONS: The CST and MIST are reliable in patients with BCT. Patients tolerated the MIST more than the CST. Better lung function and 6-MWT scores predicted the greater NOSs and greater peak HR. .

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.

Efeitos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos e na remoção de secreção de pacientes com bronquiectasia / Effects of ELTGOL and Flutter VRP1® on the dynamic and static pulmonary volumes and on the secretion clearance of patients with bronchiectasis

Contextualização: Embora a fisioterapia respiratória seja considerada fundamental para o tratamento de pacientes hipersecretivos, há poucas evidências acerca de seus efeitos fisiológicos e terapêuticos em indivíduos com bronquiectasia. Objetivos: Avaliar os efeitos fisiológicos imediatos da ELTGOL e do Flutter® nos volumes pulmonares dinâmicos e estáticos em pacientes com bronquiectasia e, secundariamente, determinar o efeito dessas técnicas na remoção de secreção brônquica. Métodos: Participaram do estudo pacientes com diagnóstico clínico e radiológico de bronquiectasia. Os pacientes foram submetidos a três intervenções de forma randomizada e com um intervalo (washout) de uma semana entre elas. Inicialmente os pacientes inalaram dois jatos de 100µcg de salbutamol. Após 5 minutos de tosse iniciais e após 5 minutos de tosse que sucederam o protocolo controle e as intervenções (ELTGOL e Flutter®), os pacientes realizaram as avaliações dos volumes pulmonares dinâmicos e estáticos por meio da espirometria e pletismografia corporal. A secreção expectorada foi coletada durante as intervenções e durante a segunda série de tosse, sendo quantificada por meio de seu peso seco. Resultados: Foram avaliados dez pacientes, dois do sexo masculino e oito do sexo feminino (média de idade de 55,9±18,1 anos). Após a utilização do Flutter® e da ELTGOL, observou-se diminuição significativa do volume residual (VR), da capacidade residual funcional (CRF) e da CPT (p<0,05). Foi eliminada maior quantidade de secreção pulmonar durante a ELTGOL em comparação com o Controle e o Flutter®. Conclusão: O Flutter VRP1® e a técnica ELTGOL reduziram a hiperinsuflação pulmonar a curto prazo, porém apenas o ELTGOL aumentou a eliminação de secreção pulmonar de pacientes com bronquiectasia.

Background: Although respiratory physical therapy is considered fundamental in the treatment of hypersecretive patients, there is little evidence of its physiological and therapeutic effects in bronchiectasis patients. Objective: To evaluate the acute physiological effects of ELTGOL and Flutter VRP1® in dynamic and static lung volumes in patients with bronchiectasis and, secondarily, to study the effect of these techniques in sputum elimination. Methods: Patients with clinical and radiological diagnosis of bronchiectasis were included. Patients underwent three interventions in a randomized order and with a one-week washout interval between them. Before all interventions patients inhaled two puffs of 100 mcg of salbutamol. There was a cough period of five minutes before and after the control protocol and the interventions (ELTGOL and Flutter VRP1®). After each cough series patients underwent assessments of dynamic and static lung volumes by spirometry and plethysmography. The expectorated secretions were collected during the interventions and during the second cough series, and quantified by its dry weight. Results: We studied 10 patients, two males and eight females (mean age: 55.9±18.1 years). After using Flutter VRP1®and ELTGOL there was a significant decrease in residual volume (RV), functional residual capacity (FRC) and total lung capacity (TLC) (p<0.05). There was a higher sputum production during ELTGOL compared with Control and Flutter VRP1® (p<0.05). Conclusion: The ELTGOL and Flutter VRP1® techniques acutely reduced lung hyperinflation, but only the ELTGOL increased the removal of pulmonary secretions from patients with bronchiectasis.

Bronquiectasias en pediatría, aproximación diagnóstica y tratamiento / Bronchiectasis in pediatrics, diagnosis approach and management

Las bronquiectasias son un problema de salud en países desarrollados y en vías de desarrollo. La fibrosis quística es una causa importante, si bien fuera de ésta existen causas que convierten a las bronquiectasias no relacionadas con fibrosis quística en un diagnóstico frecuente. Su diagnóstico precoz basado en la clínica y posterior confirmación radiológica es el punto de partida para determinar la etiología e instaurar un tratamiento dirigido a la causa subyacente. Para ello es menester una evaluación ordenada y sistemática. Se presenta una revisión sobre la etiología y tratamiento de las bronquiectasias, especialmentede las no relacionadas con fibrosis quística.

Adesividade e purulência de secreções respiratórias: implicações no transporte mucociliar em pacientes com bronquiectasias / Adhesiveness and purulence of respiratory secretions: implications for mucociliary transport in patients with bronchiectasis

OBJETIVO: Analisar e comparar as propriedades de transporte de secreções respiratórias, classificadas através de parâmetros selecionados, de indivíduos com bronquiectasias não secundárias à fibrose cística. MÉTODOS: Foram avaliadas amostras de muco respiratório, classificadas como com propriedades de superfície adesivas ou não adesivas, assim como com aspecto mucoide ou purulento, de 35 participantes com bronquiectasias não secundárias à fibrose cística, quanto a velocidade relativa de transporte (VRT), deslocamento em máquina simuladora de tosse (MST) e ângulo de contato (AC). Para as comparações propostas, foram utilizados modelos de ANOVA, com nível de significância estabelecido em 5 por cento. RESULTADOS: Houve uma diminuição significativa no deslocamento em MST, assim como um aumento significativo no AC, das amostras adesivas quando comparadas às não adesivas (6,52 ± 1,88 cm vs. 8,93 ± 2,81 cm e 27,08 ± 6,13º vs. 22,53 ± 5,92º, respectivamente; p < 0,05 para ambos). O mesmo ocorreu na comparação entre as amostras purulentas e mucoides (7,57 ± 0,22 cm vs. 9,04 ± 2,48 cm e 25,61 ± 6,12º vs. 21,71 ± 5,89º; p < 0,05 para ambos). Não houve diferença na VRT entre os grupos, embora os valores estivessem diminuídos, independentemente da adesividade (adesivas: 0,81 ± 0,20; não adesivas: 0,68 ± 0,24) ou do aspecto (purulentas: 0,74 ± 0,22; mucoides: 0,82 ± 0,22) das amostras. CONCLUSÕES: A secreção respiratória de pacientes com bronquiectasia apresentou uma diminuição do transporte ciliar. Maior adesividade e purulência favorecem a piora das propriedades de transporte, demonstradas pela diminuição do deslocamento em MST e pelo aumento do AC.

OBJECTIVE: To analyze and compare the transport properties of respiratory secretions, classified by selected parameters, in individuals with bronchiectasis unrelated to cystic fibrosis. METHODS: We collected mucus samples from 35 individuals with bronchiectasis unrelated to cystic fibrosis. The samples were first classified by their surface properties (adhesive or nonadhesive), as well as by their aspect (mucoid or purulent). We then tested the samples regarding relative transport velocity (RTV), displacement in a simulated cough machine (SCM), and contact angle (CA). For the proposed comparisons, we used ANOVA models, with a level of significance set at 5 percent. RESULTS: In comparison with nonadhesive samples, adhesive samples showed significantly less displacement in the SCM, as well as a significantly higher CA (6.52 ± 1.88 cm vs. 8.93 ± 2.81 cm and 27.08 ± 6.13º vs. 22.53 ± 5.92º, respectively; p < 0.05 for both). The same was true in the comparison between purulent and mucoid samples (7.57 ± 0.22 cm vs. 9.04 ± 2.48 cm and 25.61 ± 6.12º vs. 21.71 ± 5.89º; p < 0.05 for both). There were no significant differences in RTV among the groups of samples, although the values were low regardless of the surface properties (adhesive: 0.81 ± 0.20; nonadhesive: 0.68 ± 0.24) or the aspect (purulent: 0.74 ± 0.22; mucoid: CONCLUSIONS: The respiratory secretions of patients with bronchiectasis showed decreased mucociliary transport. Increased adhesiveness and purulence cause the worsening of transport properties, as demonstrated by the lesser displacement in the SCM and the higher CA.

Analysis of the sputum and inflammatory alterations of the airways in patients with common variable immunodeficiency and bronchiectasis

INTRODUCTION: Common variable immunodeficiency is characterized by defective antibody production and recurrent pulmonary infections. Intravenous immunoglobulin is the treatment of choice, but the effects of Intravenous immunoglobulin on pulmonary defense mechanisms are poorly understood. OBJECTIVE: The aim of this study was to verify the impact of intravenous immunoglobulin on the physical properties of the sputum and on inflammatory alterations in the airways of patients with Common variable immunodeficiency associated with bronchiectasis. METHOD: The present study analyzed sputum physical properties, exhaled NO, inflammatory cells in the sputum, and IG titers in 7 patients with Common variable immunodeficiency and bronchiectasis with secretion, immediately before and 15 days after Intravenous immunoglobulin. A group of 6 patients with Common variable immunodeficiency and bronchiectasis but no sputum was also studied for comparison of the basal IgG level and blood count. The 13 patients were young (age=36±17 years) and comprised predominantly of females (n=11). RESULTS: Patients with secretion presented significantly decreased IgG and IgM levels. Intravenous immunoglobulin was associated with a significant decrease in exhaled NO (54.7 vs. 40.1 ppb, p<0.05), sputum inflammatory cell counts (28.7 vs. 14.6 cells/mm³, p<0.05), and a significant increase in respiratory mucus transportability by cough (42.5 vs. 65.0 mm, p < 0.05). CONCLUSION: We concluded that immunoglobulin administration in Common variable immunodeficiency patients results in significant improvement in indexes of inflammation of the airways with improvement in the transportability of the respiratory mucus by cough.

Bronquiectasias no asociadas a fibrosis quística / Non-cystic fibrosis bronchiectasis

Las bronquiectasias no asociadas a fibrosis quística (FQ) son causa frecuente de morbimortalidad de acuerdo a la severidad de la patología subyacente. Su diagnóstico precoz en base a la clínica y el scanner, toda vez que se haya descartado una FQ, es clave para el manejo oportuno y sistemático de los distintos tipos asociados a las diversas etiologías que se menciona. Se han realizado avances en el estudio genético de los factores intrínsecos presentes en poblaciones específicas. El manejo adecuado de la patología de base, cuando se descubre, no siempre resulta exitoso y el tratamiento definitivo es motivo de investigación. Por ahora, el uso de antibióticos sistémicos e inhalados más fisioterapia son las únicas herramientas que han demostrado eficacia en mejorar síntomas y calidad de vida en la mayoría de los casos. Se presenta una revisión actualizada de las causas de bronquiectasias no relacionadas con FQ, incidencia y prevalencia, aspectos clínicos más relevantes, junto a las evidencias y controversias sobre su tratamiento.

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.

Reduced exercise capacity in non-cystic fibrosis bronchiectasis.

OBJECTIVE: Bronchiectasis not due to cystic fibrosis is usually a consequence of severe bacterial or tuberculous infection of the lungs, which is commonly seen in children in developing countries. Our aim was to study its functional sequelae and affect on work capacity in children. METHODS: Seventeen children (7-17 years of age) with clinical and radiological evidence of bronchiectasis of one or both lungs were studied at the Cardiopulmonary Unit of the Tuberculosis Research Centre. Pulmonary function tests including spirometry and lung volume measurements were performed. Incremental exercise stress test was done on a treadmill, and ventilatory and cardiac parameters were monitored. Control values were taken from a previous study. RESULTS: Children with bronchiectasis had lower forced vital capacity (FVC) (1.1 + 0.4 L versus 1.5 + 0.4 L, p = 0.003) and FEV1 (0.95 +/- 0.2 L versus 1.4 +/- 0.3 L, p < 0.002) compared to age- and sex-matched healthy controls. The patient group had significantly higher residual lung volumes (0.7 +/- 0.3 L versus 0.4 + 0.1 L, p < 0.02). At maximal exercise, they had lower aerobic capacity (28 +/- 6 ml/min/kg versus 38 +/- 5 ml/min/kg, p < 0.0001) and maximal ventilation (24 +/- 8 L/min versus 39 +/- 10 L/min, p < 0.001). At maximal exercise, while none of the controls desaturated, oxygen saturation fell below 88% in eight of 17 patients. CONCLUSION: The findings show that children and adolescents with non-cystic fibrosis bronchiectasis have abnormal pulmonary function and reduced exercise capacity. This is likely to interfere with their life as well as future work capacity. Efforts should be made to minimize lung damage in childhood by ensuring early diagnosis and instituting appropriate treatment of respiratory infections.