Spectrum of Pulmonary Fibrosis from Interstitial Lung Abnormality to Usual Interstitial Pneumonia: Importance of Identification and Quantification of Traction Bronchiectasis in Patient Management.

Following the introduction of a novel pathological concept of usual interstitial pneumonia (UIP) by Liebow and Carrington in 1969, diffuse interstitial pneumonia has evolved into UIP, nonspecific interstitial pneumonia (NSIP), and interstitial lung abnormality (ILA); the histopathological and CT findings of these conditions reflect the required multidisciplinary team approach, involving pulmonologists, radiologists, and pathologists, for their diagnosis and management. Concomitantly, traction bronchiectasis and bronchiolectasis have been recognized as the most persistent and important indices of the severity and prognosis of fibrotic lung diseases. The traction bronchiectasis index (TBI) can stratify the prognoses of patients with ILAs. In this review, the evolutionary concepts of UIP, NSIP, and ILAs are summarized in tables and figures, with a demonstration of the correlation between CT findings and pathologic evaluation. The CT-based UIP score is being proposed to facilitate a better understanding of the spectrum of pulmonary fibrosis, from ILAs to UIP, with emphasis on traction bronchiectasis/bronchiolectasis.

Is bronchiectasis really a disease?

The definition of a disease requires that distinguishing signs and symptoms are present that are common, and that the constellation of signs and symptoms differentiate the condition from other causes. In bronchiectasis, anatomical changes, airways inflammation and airway infection are the distinguishing features that are common to this disease. However, bronchiectasis is a heterogenous disease: signs and symptoms are shared with other airway diseases, there are multiple aetiologies and certain phenotypes of bronchiectasis have distinct clinical and laboratory features that are not common to all people with bronchiectasis. Furthermore, response to therapeutic interventions in clinical trials is not uniform. The concept of bronchiectasis as a treatable trait has been suggested, but this may be too restrictive in view of the heterogeneity of bronchiectasis. It is our opinion that bronchiectasis should be defined as a disease in its own right, but one that shares several pathophysiological features and "treatable traits" with other airway diseases. These traits define the large heterogeneity in the pathogenesis and clinical features and suggest a more targeted approach to therapy.

The clinical characteristics and prognosis of ABPA are closely related to the mucus plugs in central bronchiectasis.

INTRODUCTION: The characteristics of Allergic Bronchopulmonary Aspergillosis (ABPA) based on its radiological classification is still unclear. OBJECTIVES: To investigate the clinical significances of ABPA patients with central bronchiectasis (ABPA-CB) by different radiological classifications of mucus plugs. METHODS: ABPA-CB patients from a pulmonary hospital between 2008 and 2015 were retrospectively included and analysed. According to the chest imaging in their first visit to physician, the ABPA-CB patients were divided into two groups based on the presence of high-attenuation mucus (HAM) or low-attenuation mucus (LAM). The primary endpoint was ABPA relapse within 1 year since the glucocorticoid withdrawal. The relationship between the imaging findings and the clinical prognosis was illuminated. RESULTS: A total of 125 ABPA patients were analysed in this study. Compared to the LAM group, the HAM group presented higher blood eosinophil cells counts, higher rates of Aspergillus detection isolated in sputum and expectoration of brownish-black mucus plugs, more affected lobes and segments, poorer pulmonary function and higher rate of relapse. CONCLUSIONS: The clinical characteristics and prognosis of ABPA-CB patients are closely related to its radiological phenotype of mucus plugs in the central bronchiectasis. Clinicians should promote a diversity of personalized treatments for different patients with different radiological characteristics.

Prognostic Value of Frequent Exacerbations in Bronchiectasis: The Relationship With Disease Severity.

INTRODUCTION: Bronchiectasis is a very heterogeneous disease but some homogeneous groups with similar clinical characteristics and prognosis have been identified. Exacerbations have been shown to have a negative impact on the natural history of bronchiectasis. The objective of this study was to identify the definition and characteristics of the "frequent exacerbator patient" with the best prognostic value and its relationship with the severity of bronchiectasis. METHODS: A historical cohort of 651 patients diagnosed with bronchiectasis was included. They had all received 5 years of follow-up since their radiological diagnosis. Exacerbation was defined as a worsening of the symptoms derived from bronchiectasis that required antibiotic treatment. The main outcome was all-cause mortality at the end of follow-up. RESULTS: The mean age was 48.2 (16) years (32.9% males). 39.8% had chronic infection by Pseudomonas aeruginosa. Mean BSI, FACED, and E-FACED were 7 (4.12), 2.36 (1.68), and 2.89 (2.03), respectively. There were 95 deaths during follow-up. The definition of the "frequent exacerbator patient" that presented the greatest predictive power for mortality was based on at least two exacerbations/year or one hospitalization/year (23.3% of patients; AUC-ROC: 0.75 [95% CI: 0.69-0.81]). Its predictive power was independent of the patient's initial severity. The clinical characteristics of the frequent exacerbator patient according to this definition varied according to the initial severity of bronchiectasis, presence of systemic inflammation, and treatment. CONCLUSIONS: The combination of two exacerbations or one hospitalization per year is the definition of frequent exacerbator patient that has the best predictive value of mortality independent of the initial severity of bronchiectasis.

Papel de los estudios de imagen en el diagnóstico etiológico de las bronquiectasias: la distribución es la clave / The role of imaging in the diagnosis of bronchiectasis: the key is in the distribution

La patología de las vías respiratorias de medio calibre (bronquios segmentarios y subsegmentarios) es común y se presenta con síntomas respiratorios poco específicos, como tos, infecciones de repetición y en ocasiones hemoptisis. La dilatación permanente del árbol bronquial se conoce como «bronquiectasia» y representa un reto diagnóstico. El análisis de la distribución regional de las bronquiectasias en los diferentes lóbulos pulmonares es la guía diagnóstica más útil. El objetivo de este trabajo es describir los hallazgos de imagen de las bronquiectasias y sus diferentes tipos, revisar las situaciones más comunes y proponer un algoritmo diagnóstico basado en su distribución anatómica. Las bronquiectasias son un hallazgo frecuente, resultado de un amplio espectro de enfermedades. Los estudios de imagen desempeñan un papel esencial en su detección, clasificación y orientación diagnóstica hacia la patología subyacente (AU)

Diseases that involve the medium caliber airways (segmental and subsegmental bronchi) are common and present clinically with nonspecific respiratory symptoms such as cough, recurrent respiratory infections and occasionally, hemoptysis. The abnormal and irreversible dilation of bronchi is known as "bronchiectasis". The diagnosis can be challenging and the analysis of the regional distribution of the bronchiectasis is the most useful diagnostic guide. The objective of this manuscript is to describe the main imaging findings of bronchiectasis and their classification, review the diseases that most commonly present with this abnormality, and provide an approach to the diagnosis based on their imaging appearance and anatomic distribution. Bronchiectasis is a frequent finding that may result from a broad range of disorders. Imaging plays a paramount role in diagnosis, both in the detection and classification, and in the diagnosis of the underlying pathology (AU)

Normativa sobre la valoración y el diagnóstico de las bronquiectasias en el adulto / Spanish Guidelines on the Evaluation and Diagnosis of Bronchiectasis in Adults

En 2008 la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) publicó las primeras normativas del mundo sobre el diagnóstico y tratamiento de las bronquiectasias. Tras casi una década muchos han sido los avances científicos en esta enfermedad, no solo en sus aspectos terapéuticos, sino también en su valoración y diagnóstico. Por ello, estas nuevas normativas sobre la valoración y diagnóstico de las bronquiectasias tratan de ofrecer al lector una actualización del conocimiento científico sobre las bronquiectasias basándose en un estricto procedimiento metodológico que asegura la calidad del contenido de las mismas, y en una amplia cantidad de información online que incluye abundante bibliografía. Estas normativas recogen desde una definición consensuada de bronquiectasias hasta la valoración de la historia natural y del pronóstico de la enfermedad. Se tratan los temas de mayor interés y algunos novedosos, como epidemiología y costes económicos de las bronquiectasias, aspectos fisiopatológicos, etiología (haciendo especial énfasis en la relación con otras enfermedades de la vía aérea como la enfermedad pulmonar obstructiva crónica y el asma), aspectos clínico-funcionales, medición de la calidad de vida, diagnóstico y valoración radiológica, algoritmo diagnóstico, aspectos microbiológicos (incluyendo la definición consensuada de conceptos clave como el de erradicación bacteriana o infección bronquial crónica), así como la valoración de la gravedad y el pronóstico de la enfermedad mediante el uso de las nuevas herramientas multidimensionales publicadas (AU)

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest scientific knowledge on bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure the quality of content, and are linked to a large amount of online information that includes a wealth of references. These guidelines cover aspects ranging from a consensual definition of bronchiectasis to an evaluation of the natural course and prognosis of the disease. The topics of greatest interest and some new areas are addressed, including epidemiology and economic costs of bronchiectasis, pathophysiological aspects, the causes (placing particular emphasis on the relationship with other airway diseases such as chronic obstructive pulmonary disease and asthma), clinical and functional aspects, measurement of quality of life, radiological diagnosis and assessment, diagnostic algorithms, microbiological aspects (including the definition of key concepts, such as bacterial eradication or chronic bronchial infection), and the evaluation of severity and disease prognosis using recently published multidimensional too (AU)

Normativa sobre el tratamiento de las bronquiectasias en el adulto / Spanish Guidelines on the Evaluation and Diagnosis of Bronchiectasis in Adults

En 2008, la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) publicó las primeras normativas del mundo sobre el diagnóstico y tratamiento de las bronquiectasias. Tras casi una década, muchos han sido los avances científicos en esta enfermedad, tanto en sus aspectos terapéuticos como en su valoración y diagnóstico. Por ello estas nuevas normativas sobre el tratamiento de las bronquiectasias en el adulto tratan de ofrecer al lector una actualización del conocimiento científico sobre las posibilidades terapéuticas en bronquiectasias, basándose en un estricto procedimiento metodológico que asegura la calidad del contenido de la misma, y en una amplia cantidad de información online que incluye abundante bibliografía. En estas normativas se ha enfocado el tratamiento de las bronquiectasias desde un punto de vista tanto multidisciplinar, que implica las especialidades y escalones asistenciales involucrados, como multidimensional que incluye todos y cada uno de los aspectos que definen a la enfermedad. Así, se establecen recomendaciones basadas en una exhaustiva revisión de la evidencia sobre los tratamientos de la etiología, de la infección bronquial en sus diferentes formas de presentación y con las diferentes terapias existentes, de la inflamación bronquial y de la obstrucción al flujo aéreo. Se revisan los aspectos nutricionales, el manejo de las secreciones, el entrenamiento muscular, el manejo de las complicaciones y comorbilidades, la profilaxis de la infección, los aspectos educacionales, el manejo del paciente en el domicilio, el tratamiento quirúrgico, las agudizaciones y el seguimiento de los pacientes (AU)

In 2008, the Spanish Society of Pulmonology (SEPAR) published the first guidelines in the world on the diagnosis and treatment of bronchiectasis. Almost 10 years later, considerable scientific advances have been made in both the treatment and the evaluation and diagnosis of this disease, and the original guidelines have been updated to include the latest therapies available for bronchiectasis. These new recommendations have been drafted following a strict methodological process designed to ensure quality of content, and are linked to a large amount of online information that includes a wealth of references. The guidelines are focused on the treatment of bronchiectasis from both a multidisciplinary perspective, including specialty areas and the different healthcare levels involved, and a multidimensional perspective, including a comprehensive overview of the specific aspects of the disease. A series of recommendations have been drawn up, based on an in-depth review of the evidence for treatment of the underlying etiology, the bronchial infection in its different forms of presentation using existing therapies, bronchial inflammation, and airflow obstruction. Nutritional aspects, management of secretions, muscle training, management of complications and comorbidities, infection prophylaxis, patient education, home care, surgery, exacerbations, and patient follow-up are addressed (AU)

Tracheobronchomegaly in Intubated Ventilation of ARDS / Traqueobroncomegalia en intubación y ventilación mecánica en un caso de síndrome de dificultad respiratoria aguda (SDRA)

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The Multiple Faces of Non-Cystic Fibrosis Bronchiectasis. A Cluster Analysis Approach.

RATIONALE: The clinical presentation and prognosis of non-cystic fibrosis bronchiectasis are both very heterogeneous. OBJECTIVES: To identify different clinical phenotypes for non-cystic fibrosis bronchiectasis and their impact on prognosis. METHODS: Using a standardized protocol, we conducted a multicenter observational cohort study at six Spanish centers with patients diagnosed with non-cystic fibrosis bronchiectasis before December 31, 2005, with a 5-year follow-up from the bronchiectasis diagnosis. A cluster analysis was used to classify the patients into homogeneous groups by means of significant variables corresponding to different aspects of bronchiectasis (clinical phenotypes): age, sex, body mass index, smoking habit, dyspnea, macroscopic appearance of sputum, number of exacerbations, chronic colonization with Pseudomonas aeruginosa, FEV1, number of pulmonary lobes affected, idiopathic bronchiectasis, and associated chronic obstructive pulmonary disease. Survival analysis (Kaplan-Meier method and log-rank test) was used to evaluate the comparative survival of the different subgroups. MEASUREMENTS AND MAIN RESULTS: A total of 468 patients with a mean age of 63 (15.9) years were analyzed. Of these, 58% were females, 39.7% had idiopathic bronchiectasis, and 29.3% presented with chronic Pseudomonas aeruginosa colonization. Cluster analysis showed four clinical phenotypes: (1) younger women with mild disease, (2) older women with mild disease, (3) older patients with severe disease who had frequent exacerbations, and (4) older patients with severe disease who did not have frequent exacerbations. The follow-up period was 54 months, during which there were 95 deaths. Mortality was low in the first and second groups (3.9% and 7.6%, respectively) and high for the third (37%) and fourth (40.8%) groups. The third cluster had a higher proportion of respiratory deaths than the fourth (77.8% vs. 34.4%; P < 0.001). CONCLUSIONS: Using cluster analysis, it is possible to separate patients with bronchiectasis into distinct clinical phenotypes with different prognoses.

Correlation between Bhalla score and spirometry in children and adolescents with cystic fibrosis.

OBJECTIVE: To correlate the findings of high resolution computed tomography of the chest based on the Bhalla score with the clinical data and spirometry in children and adolescents with cystic fibrosis, and to study the concordance between two radiologists for the Bhalla score and its categories. METHODS: We evaluated the medical records of 23 patients from the outpatient clinic. The items evaluated included age, weight, height, height/age Z-score, weight/age Z-score, body mass index (BMI), O2 saturation, spirometry and Bhalla score. RESULTS: The patients had a mean age of 17.4 years ± 5.7 years, with fifteen females and eight males. There was good correlation between Bhalla score and spirometry (FVC-r =0.718, p<0.001; FEV1-r=0.830, p<0.001; FEF25-75%-r =0.786, p<0.001; FEV1/FVC-r=0.714, p<0.001). It was also noted that some patients with FEF25-75%> 70% already had changes in their final Bhalla score. In the analysis of the concordance between the examiners a Kappa coefficient of 0.81 (p <0.001) was found, and an intraclass correlation coefficient of 0.98. CONCLUSION: A good correlation between Bhalla scores with spirometry confirmed its usefulness in evaluating and monitoring patients with cystic fibrosis, given it can be used both in patients who are unable to perform spirometry as well as for a pooled analysis of the two examinations since the HRCT scans show early changes in patients with normal function tests.

Correlation between Bhalla score and spirometry in children and adolescents with Cystic Fibrosis / Correlação entre escore de Bhalla e espirometria em crianças e adolescentes com fibrose cística

Objective: to correlate the findings of high resolution computed tomography of the chest based on the Bhalla score with the clinical data and spirometry in children and adolescents with cystic fibrosis, and to study the concordance between two radiologists for the Bhalla score and its categories. Methods: we evaluated the medical records of 23 patients from the outpatient clinic. The items evaluated included age, weight, height, height/age Z-score, weight/age Z-score, body mass index (BMI), O2 saturation, spirometry and Bhalla score. Results: the patients had a mean age of 17.4 years ± 5.7 years, with fifteen females and eight males. There was good correlation between Bhalla score and spirometry (FVC-r =0.718, p<0.001; FEV1-r=0.830, p<0.001; FEF25-75%-r =0.786, p<0.001; FEV1/FVC-r=0.714, p<0.001). It was also noted that some patients with FEF25-75%> 70% already had changes in their final Bhalla score. In the analysis of the concordance between the examiners a Kappa coefficient of 0.81 (p <0.001) was found, and an intraclass correlation coefficient of 0.98. Conclusion: a good correlation between Bhalla scores with spirometry confirmed its usefulness in evaluating and monitoring patients with cystic fibrosis, given it can be used both in patients who are unable to perform spirometry as well as for a pooled analysis of the two examinations since the HRCT scans show early changes in patients with normal function tests. .

Objetivo: correlacionar os achados da tomografia computadorizada de alta resolução (TCAR) do tórax, com base no escore de Bhalla, com os dados clínicos e a espirometria em crianças e adolescentes com fibrose cística (FC), além de estudar a concordância entre dois médicos radiologistas para o escore de Bhalla e suas categorias. Métodos: foram avaliados os prontuários e os exames de 23 pacientes do ambulatório. Os itens avaliados foram idade, peso, altura, escore Z altura/idade, escore Z peso/ idade, índice de massa corpórea (IMC), saturação de O2, espirometria e escore de Bhalla. Resultados: os pacientes avaliados tinham média de idade de 17,4±5,7 anos, sendo 15 do sexo feminino e 8 do sexo masculino. Houve boa correlação entre o escore de Bhalla e a espirometria (CVF-r = 0,718, p < 0,001; VEF1-r = 0,830, p < 0,001; FEF 25-75%-r = 0,786, p < 0,001; VEF1/ CVF-r = 0,714, p < 0,001). Nota-se, ainda, que alguns pacientes com FEF 25-75% > 70% já apresentavam alterações na nota final do escore de Bhalla. Na análise da concordância entre os examinadores, foi encontrado coeficiente kappa de 0,81 (p < 0,001) e coeficiente de correlação intraclasse de 0,98. Conclusão: a boa correlação do escore de Bhalla com as provas de função pulmonar confirma a sua utilidade na avaliação e no acompanhamento dos pacientes com FC, podendo ser utilizado tanto para pacientes que são incapazes de realizar a espirometria quanto para uma análise em conjunto dos dois exames, uma vez que a TCAR mostra alterações precoces em pacientes com espirometrias normais. .

[Understanding the classification, physiopathology and the diagnostic radiology of bronchiectasis]. / Entendendo a classifi cação, a fi siopatologia e o diagnóstico radiológico das bronquiectasias.

Bronchiectasis is defined as an abnormal persistent bronchial dilatation usually associated with inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Computed tomography is now the diagnostic modality of choice and may also contribute to clinical management, suggesting some etiologic causes. We highlight developments in classification, physiopathology and radiology of this debilitating disease.

[Diagnosis and treatment of bronchiectasis. Spanish Society of Pneumology and Thoracic Surgery]. / Diagnóstico y tratamiento de las bronquiectasias. SEPAR.

Bronchiectasis is the end result of several different diseases that share principles of management. The clinical course usually involves chronic bronchial infection and inflammation, which are associated with progression. The cause of bronchiectasis should always be investigated, particularly when it can be treated. We recommend evaluating etiology, symptoms, bronchial colonization and infection, respiratory function, inflammation, structural damage, nutritional status, and quality of life in order to assess severity and to monitor clinical course. Care should be supervised by specialized units, at least in cases of chronic bronchial infection, recurrent exacerbations, or when there is a cause that is likely to respond to treatment. Improving symptoms and halting progression are the goals of management, which is based on treatment of the underlying cause and of acute or chronic infections and on the drainage of secretions. Complications that arise must also be treated. Antibiotic prescription is guided by how well infection is being controlled, and this is indicated by the color of sputum and a reduction in the number of exacerbations. We recommend inhaled antibiotics in cases of chronic bronchial infection that does not respond to oral antibiotics, when these cause side effects, or when the cause is Pseudomonas species or other bacteria resistant to oral antibiotics. Inhaled administration is also advisable to treat initial colonization by Pseudomonas species.

Surgical results for bronchiectasis based on hemodynamic (functional and morphologic) classification.

OBJECTIVE: This study was a prospective evaluation of surgical indications and outcomes for unilateral and bilateral bronchiectasis according to hemodynamic (functional and morphologic) classification. METHODS: Between January 1998 and January 2004, the morphologic features (cystic versus cylindric) by chest computed tomography and the hemodynamic features (perfused versus nonperfused) by lung ventilation/perfusion scan were determined in 66 patients with bronchiectasis (53 unilateral and 13 bilateral). The indication for surgical resection in both groups was the presence of localized areas of cystic, nonperfused bronchiectasis. RESULTS: In the unilateral bronchiectasis group, there were 28 female and 25 male patients with an average age of 37.5 +/- 3.8 years (range 6-40 years). Pneumonectomy was performed in 10 cases (8 left and 2 right), and lobectomy or bilobectomy was performed in 43. In the bilateral group, there were 7 male and 6 female patients with an average age of 42 +/- 5.4 years (range 9-55 years). Pneumonectomy was performed in 2 cases, lobectomy in 5, and bilateral staged lobectomy in 6. There was 1 postoperative death (1.5%), and morbidity was 18% (12 patients). Four patients required reexploration for bleeding, 4 had prolonged air leak develop, 3 acquired pulmonary infections, and 1 had localized empyema develop. During a mean follow-up of 52 months (range 24-82 months), 48 patients were considered cured (73%) and 17 had symptomatic improvement (26%). Pseudomonas infection and underlying chronic obstructive airway disease were poor prognostic factors (P < .05). CONCLUSION: The hemodynamic (functional and morphologic) classification provides an accurate functional classification for bronchiectasis. Its application in determining the indications and extent of surgical resection is superior to morphologic classification alone. Curative resection can be achieved in both unilateral and bilateral bronchiectasis with acceptable morbidity.

Mycobacterium avium complex pulmonary disease in immunocompetent patients.

Mycobacterium avium complex is becoming increasingly recognized as one of the most common mycobacterial pathogens in humans. It is rapidly becoming a significant cause of pulmonary disease even in those with an intact immunity. In 1997, the American Thoracic Society published recommendations for the diagnosis and treatment of nontuberculous mycobacteria. On the basis of the authors' clinical experience of the myriad presentations of pulmonary Mycobacterium avium complex disease in an immunocompetent host, a clinical classification is proposed. The current data are summarized, and a practical approach to management of the various pulmonary forms of the disease is provided.

[Typing of bronchiectasis according to syndrome differentiation].

OBJECTIVE: To study the general law of typing of bronchiectasis according to syndrome differentiation. METHODS: We collected the symptoms, conditions of tongue and pulse in patients of bronchiectasis, using frequencies procedure, discriminant analysis and K-means cluster analysis in SPSS statistical software as research medium. RESULTS: Five hundred and sixty three patients with bronchiectasis were studied. It suggested that accumulation of phlegm-heat in the lungs (45.65%), liver fire attacking the lungs (24.51%), asthenia of pulmonosplenic qi (22.38%), asthenia of both qi and yin (7.46%) were the main types. CONCLUSION: Clinical epidemiology provided scientific basis for further studying of the typing of bronchiectasis according to syndrome differentiation. Building up differentiation of syndromes through differentiation and analysis of main symptoms can be used in clinical diagnosis.