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.

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.

Mild, moderate, and severe forms of allergic bronchopulmonary aspergillosis: a clinical and serologic evaluation.

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity disorder induced by Aspergillus species colonizing the bronchial tree. There are patients with asthma who fulfill the diagnostic criteria of ABPA by serologic evaluation (specific IgE/IgG to Aspergillus fumigatus), bronchography, CT, and or conventional linear tomography. OBJECTIVE: To identify different forms of ABPA based on various diagnostic criteria. METHODS: Eighteen patients with asthma fulfilling the criteria of ABPA were evaluated in the present study. Six patients each received a diagnosis of ABPA serologic positive (ABPA-S), ABPA with central bronchiectasis (ABPA-CB), and ABPA with central bronchiectasis and other radiologic features (ABPA-CB-ORF). RESULTS: The spirometric changes in the ABPA-S group (group 1) were mild, in the ABPA-CB group (group 2) were moderate, and in the ABPA-CB-ORF group (group 3) were severe. Absolute eosinophil count was raised in each group but was maximum (1,233 micro L) in severe form of disease (group 3). Specific IgE against A fumigatus was raised in each group, and the maximum was 47.91 IU/mL in ABPA-CB-ORF. CT scan findings of the ABPA-S group were normal without central bronchiectasis. The exacerbation in symptoms was maximum in group 3 compared to other groups. CONCLUSION: The present observations suggest that ABPA includes mild (ABPA-S), moderate (ABPA-CB), and severe (ABPA-CB-ORF) forms of disease. It is recommended, therefore, that the disease should be diagnosed early, treated at the mild form of disease (ABPA-S), and prevented from leading to ABPA-CB or ABPA-CB-ORF.

Diagnosis of bronchiectasis with multislice spiral CT: accuracy of 3-mm-thick structured sections.

The aim of this study was to evaluate the accuracy of 3-mm-thick reconstructed sections in the diagnosis of bronchiectasis with multislice CT (MSCT). Forty consecutive patients suspected of bronchiectasis (23 females, 17 males; mean age 51 years) underwent MSCT of the entire thorax with a 4x1-mm collimation (120 kV, 0.5 s/rotation, 80 mAs/slice) and a pitch of 1.75. From each data set (mean z-axis coverage: 257 mm; mean duration: 21 s), two series of images were systematically generated: 1-mm (group 1) and 3-mm (group 2)-thick reconstructed scans. Both series of images were obtained at 10-mm intervals and reconstructed with a high-spatial-frequency algorithm. Two observers independently analyzed the presence of bronchiectasis and associated abnormalities in group-1 and group-2 lung images. No significant difference between group 1 and group 2 was found in: (a) the detection of bronchiectasis, identified in 24 patients (60%) in group 1 and in 23 patients (57.5%) in group 2 ( p=0.08); (b) the evaluation of the extent of bronchiectasis, identifying focal bronchiectasis in 10 patients (25%) in group 1 and 7 patients (17.5%) in group 2 ( p=0.39) and multifocal bronchiectasis in 16 patients (40%) in both groups; (c) the characterisation of bronchiectasis (cylindral bronchiectasis: group 1, n=24, 60%; group 2, n=21, 53%, p=0.08); varicose bronchiectasis: group 1, n=5, 12.5%; group 2, n=6, 15%, p=0.56); and cystic bronchiectasis: group 1, n=2, 5%; group 2, n=2, 5%). Apart from the identification of abnormal bronchial wall thickening (group 2, n=35, 87.5%, vs group 1, n=31, 77.5%, p<0.05), recognition of associated bronchopulmonary anomalies did not differ between the two groups. This study demonstrates a comparable accuracy of the 3- and 1-mm-thick reconstructed scans in the detection and characterization of bronchiectasis. These results suggest the potential usefulness of 3-mm-thick scans generated from 4x2.5-mm acquisitions in the screening of bronchiectasis, which would allow a 20% radiation dose reduction compared with the present investigation.

[Bronchiectasis].

Obstructive impairment of pulmonary function in bronchiectasis depends on the number of segments involved in the bronchiectatic process and severity of the morphologic abnormalities in the region of the dilated bronchi. There is no obstructive change in bronchiectasis with single segmental lesion without fatal cases with multiple dilatation of bronchi. Immotile cilia syndrome, Kartagener syndrome, diffuse panbronchiolitis and cystic fibrosis can result from a multitude of heredity were observed in the obstructive impairment. A case of panbronchiolitis obliterans with diffuse cystic dilatation of bronchi due to mycoplasma pneumoniae infection was presented in severe obstructive disturbance.

Current surgical therapy for bronchiectasis.

The ideal classification system for bronchiectasis continues to be debated. As an alternative to the present morphologic classification, a hemodynamic-based functional classification is proposed. This study examines the rationale for and outcome of surgery based on this classification in patients with unilateral or bilateral bronchiectasis. Between July 1987 and January 1997 the morphologic and hemodynamic features in 85 bronchiectatic patients were examined: 18 with bilateral bronchiectasis and 67 with unilateral disease. A policy of unilateral lung resection of the nonperfused bronchiectasis and preservation of the perfused type was adopted in all patients. The mean age at operation was 29.4 +/- 9.7 years (range 6-55 years) with a mean follow-up period of 45.2 +/- 21.0 months (range 2-120 months). Left-sided predominance of bronchiectasis was evident in this series both in frequency and severity. In those with unilateral disease, bronchiectasis was left-sided in 49 (73.1%) patients and right-sided in 18 (26.9%). The left lung was totally bronchiectatic in 11 (16.4%) patients and the right in 3 (4.4%). Moreover, among the patients with bilateral bronchiectasis, 14 of 18 (77.7%) patients had the left lung more severely involved. Based on the morphologic and hemodynamic features in the investigated patients, two types of bronchiectasis were recognized: a perfused type with intact pulmonary artery flow and a nonperfused type with absent pulmonary artery flow. Lobectomy was performed in 55 patients, basal segmentectomy and preservation of the apical segment in 16, and pneumonectomy in 14. There was no mortality in this series. Altogether 63 patients (74.1%) achieved excellent results, 19 (22.4%) scored good results, and 3 (3.5%) patients had not benefited from surgery at last follow-up. In the face of the general criticism of the traditional morphologic classification, the proposed classification not only predicts whether the involved lung will have a measure of respiratory function with regard to gas exchange but reflects the degree of severity of the disease process. Thus the question of which side to resect and which to preserve is defined more precisely. This classification was found to be logical, physiologically sound, and of proven benefit.