Description. 50, No. Note the nodular opacities and volume loss in the right upper lobe; the faint linear opacities represent bronchiectasis. Note that both the right and left heart borders are obscured. The number and severity of episodes of acute rejection correlate with the development of bronchiolitis obliterans; therefore, close monitoring and aggressive treatment of patients with acute rejection are paramount. 6. Habesoglu MA, Ugurlu AO, Eyuboglu FO. 52, No. Although bronchial and bronchiolar dilatation due to increased intraluminal airway pressure (such as coughing or barotrauma) can be reversible, as sometimes seen in patients with pneumonia or acute respiratory distress syndrome (14), true bronchiectasis is irreversible. Figure 8b Chronic atypical mycobacterial infection in an 80-year-old woman with a history of chronic cough and M avium-intracellulare infection. Javidan-Nejad C, Bhalla S. Bronchiectasis. 37, No. Figure 10b Lower lobe bronchiectasis likely due to chronic aspiration in a 69-year-old woman. The associated finding of dextrocardia can be seen at chest radiography in cases of Kartagener syndrome. Figure 8a Chronic atypical mycobacterial infection in an 80-year-old woman with a history of chronic cough and M avium-intracellulare infection. The three most important mechanisms that contribute to the pathogenesis of bronchiectasis are infection, airway obstruction and peribronchial fibrosis. In one series, central bronchiectasis was uncommon in all causes except allergic bronchopulmonary aspergillosis, in which it was seen in 44% of cases (25). Figure 15b Tracheobronchomegaly (Mounier-Kuhn syndrome). These appearances often coexist in a single patient, and the overlapping range of morphologic features observed in most diseases associated with bronchiectasis necessitates the use of other characteristics, such as spatial distribution, to determine the cause. The enlargement of the airways is attributable to absence or marked atrophy of the elastic fibers and smooth muscle in the wall of the trachea and main bronchi. Tree-in-bud nodules indicating bronchiolitis and airtrapping are also common (51). (b) Coronal reformatted minimum intensity projection CT image shows extensive lower lobe bronchiectasis with cylindrical morphologic features, a distribution and appearance that can be seen in cases of chronic aspiration. Upper lobe volume loss has worsened, with upward hilar retraction; overall lung volumes were not decreased because of the extensive airtrapping. Oxford University Press, USA. Bronchiolitis obliterans in the posttransplantation cohort has significant implications for mortality, with one study reporting 88.8% of deaths from terminal respiratory failure among patients who underwent transplantation and had bronchiolitis obliterans, compared with 44% among patients who underwent transplantation and did not have bronchiolitis obliterans (55). Symptoms typically include a chronic cough with mucus production. Figure 1a Bronchiectasis in a 43-year-old man with cystic fibrosis. In chronic aspiration, bilateral peripheral lower lung zone (dependent)–predominant bronchiectasis can be seen (Fig 10a, 10b). (2019) Thorax. In saccular, or cystic, bronchiectasis, involved bronchi show focal pouch-like areas of enlargement; this form is commonly seen in cystic fibrosis but can also have many other causes (1). (b) Coronal reformatted CT image obtained the same day shows focal right upper lobe volume loss with bronchial retraction, bronchial wall thickening, and mild bronchiectasis; tree-in-bud opacities representing endobronchial spread of tuberculosis are visible in the same lobe. (c) Axial CT image obtained several months after recovery in a 49-year-old woman with prior intensive care unit admission for pneumonia and acute respiratory distress syndrome shows anterior-predominant architectural distortion with bronchiectasis in the right middle lobe and lingula, a typical distribution for post-acute respiratory distress syndrome–related fibrosis. Bronchiectasis is the end result of a wide variety of processes causing chronic airway damage. 2002;225 (3): 663-72. Detection of associated basilar-predominant honeycombing can aid in making the specific diagnosis of usual interstitial pneumonia, in which case biopsy is unnecessary (42). 246 (3): 697-722. Finally, knowledge of clinical context is also required to provide the most accurate and succinct differential diagnosis. Acute exacerbations of bronchiectasis are associated with increased mortality, with decreased forced expiratory volume in 1 second and a history of smoking associated with poor outcomes (7); patients with chronic obstructive pulmonary disease are prone to prolonged exacerbations (8). 16. Chronic obstructive pulmonary disease and bronchiectasis are different but related diseases that occur separately, but can coexist, wrote Drs. Some treatments are available. In severe cases, more extensive fibrosis and bronchiectasis can occur, leading to chronic respiratory failure. CT findings in allergic bronchopulmonary aspergillosis include cystic or varicoid bronchiectasis, a tree-in-bud pattern of nodules, bronchial wall thickening, and airtrapping with a central or proximal upper lobe predominance. Figure 2b Three basic morphologic types of bronchiectasis that are recognized at CT (many patients have a combination of these). Kartagener syndrome, current data on a classical disease. Enter your email address below and we will send you your username, If the address matches an existing account you will receive an email with instructions to retrieve your username. Bronchiectasis (plural: bronchiectases) is defined as an irreversible abnormal dilatation of the bronchial tree. Treatment of frequent pulmonary infections is supportive, and the only definitive therapy is lung transplantation, which is limited by donor lung availability. 5, 9, – 11 The bronchiectasis COPD overlap syndrome (BCOS) is noteworthy given it is both common and was reported as one of the commonest associations by Quint et al. If the address matches an existing account you will receive an email with instructions to reset your password. In cases of severe bronchiectasis limited to a segment or lobe, surgical resection can be performed (12). Symptoms are frequently present for more than 6 months before diagnosis and include progressively worsening shortness of breath and nonproductive cough. This happens because of the abnormal flaccidity of the trachea, which predisposes to ineffective cough and retained secretions, resulting in bronchiectasis. (d) Coronal reformatted minimum intensity projection CT image shows bilateral symmetric upper lobe cylindrical and varicoid bronchiectasis. Shannon A. Novosad and Alan F. Barker, both of Oregon Health and Science University, in Chronic Obstructive Pulmonary Disease and Bronchiectasis, published in Current Opinion in Pulmonary Medicine. These findings are typical in Swyer-James syndrome, an asymmetric bronchiolitis obliterans usually caused by an infectious insult during lung development. Figure 1c Bronchiectasis in a 43-year-old man with cystic fibrosis. Figure 10a Lower lobe bronchiectasis likely due to chronic aspiration in a 69-year-old woman. 21. 19. The main clinical manifestation of bronchiectasis is chronic productive cough (1). A number of features are helpful in diagnosing bronchiectasis 8,9: A number of ancillary findings are also recognized: In general, the treatment of bronchiectasis is medical and involves promoting sputum clearance, using positional physiotherapy, and early and aggressive treatment of pulmonary infections. In patients with α 1 -antitrypsin deficiency who develop bronchiectasis, the distribution tends to follow the emphysematous changes, with greater severity in the lower lungs ( 49 ). In addition, a mosaic pattern of attenuation secondary to airtrapping due to obstructed bronchi and bronchioles is commonly seen. Bronchiectasis caused by primary airway disease should be differentiated from tracion bronchiectasis as a result of fibrosis. Kartagener syndrome refers to the clinical combination of situs inversus, chronic sinusitis, and bronchiectasis in a subset of patients with ciliary dyskinesia. (a) Posteroanterior chest radiograph shows upper lobe–predominant reticulonodular opacities and elevation of both hila due to upper lobe volume loss. Use of coronal and sagittal reformatted images can be helpful in establishing an apical or basal gradient. Movie 3 Mechanics of bronchiectasis. Bronchiectasis is seen as comorbidity in patients with more common respiratory diseases most notably in those with more severe asthma and chronic obstructive pulmonary disease (COPD). Other symptoms include shortness of breath, coughing up blood, and chest pain. Diffuse ground-glass opacities are the dominant features in nonspecific interstitial pneumonia and are associated with a homogeneous pattern of cellular pneumonitis at pathologic examination (42,44). Figure 15a Tracheobronchomegaly (Mounier-Kuhn syndrome). Fenlon HM, Doran M, Sant SM et-al. (b) Axial CT image through the mid lungs shows the cardiac apex on the right and cylindrical and cystic bronchiectasis in the right middle lobe and lingula. (c, d) Axial (c) and coronal reformatted (d) minimum intensity projection CT images obtained 2 years later (at 26 years of age) show extensive cystic and varicoid bronchiectasis in the upper lobes. 1, Revue des Maladies Respiratoires Actualités, Vol. 147, 18 May 2017 | European Radiology, Vol. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Bronchiectasis is more common and severe where emphysema is seen and may add to the severity of the disease and worse health status. (c) Axial CT image shows the signet ring sign of bronchiectasis, with the dilated bronchus representing the “ring” and the adjacent smaller artery representing the “jewel” on the ring. It is predominantly a disease of middle to late life owing to the cumulative effect of smoking and other environmental risk factors. Figure 3b Bronchiolectasis, which is dilatation of the bronchioles (the noncartilaginous distal small airways) often seen in pulmonary fibrosis, aspiration, and recurrent infection. However, bronchiectasis in graft-versus-host disease has also recently been associated with “nonclassifiable interstitial pneumonia,” characterized by peribronchovascular fibrosis at histologic examination and traction bronchiectasis, reticulation, and peribronchovascular ground-glass opacities at CT (58). Emphysema and bronchiectasis in COPD patients with previous pulmonary tuberculosis: computed tomography features and clinical implications. Imaging of Bronchiectasis. Tram-track opacities are seen in cylindrical bronchiectasis, and air-fluid levels may be seen in cystic bronchiectasis. Weissleder R, Wittenberg J, Harisinghani MG et-al. ■ Describe the pathophysiologic characteristics of bronchiectasis. A challenging question, are they reversible? Rationale and objectives: Bronchiectasis (BE) is associated with chronic obstructive pulmonary disease (COPD), but emphysema and small airways disease, main pathologic features of COPD, have been sparsely studied in BE. Expiratory imaging can increase sensitivity for airtrapping by accentuating the contrast between the high attenuation seen in normal lung and low attenuation indicating airtrapping. One series revealed that the presence of more than 32% airtrapping provided 87.5% sensitivity and specificity for the diagnosis of bronchiolitis obliterans. Figure 13a Kartagener syndrome in a 52-year-old man. differential diagnoses of airspace opacification, presence of non-lepidic patterns such as acinar, papillary, solid, or micropapillary, myofibroblastic stroma associated with invasive tumor cells. Thorax 1971). Collins J, Stern EJ. Figure 7a Tuberculosis in a 29-year-old man with mild weight loss. Figure 18b Swyer-James syndrome with asymmetric bronchiectasis in an entire lung. (2005) ISBN:0198569785. Irregularity of the tracheal wall is attributable to laxity of the tracheal cartilage in this disorder. 4. It contains the amount of bronchial dilatation and the number of bronchopulmonary segments with emphysema, resulting in an outcome between 0 and 5. Bronchiectasis: accuracy of high-resolution CT in the differentiation of specific diseases. In one series, longer exposure to higher inspiratory pressures and high fraction of inspired oxygen correlated with the extent and severity of parenchymal changes noted at imaging (39). Figure 19 Bronchiolitis obliterans with diffuse bronchiectasis several years after bilateral lung transplantation in a 67-year-old man. A deleterious cytokine cascade occurs, with recruitment and stimulation of immune cells. Focal bronchiectasis can be secondary to a variety of causes, with the differential diagnosis including tumor, endobronchial foreign body, or infection, such as in the case of tuberculosis. Among the lung parenchyma changes, a combination of bronchiectasis and emphysema is unique to this syndrome. AJR Am J Roentgenol. The authors will also present an approach emphasizing the distribution (apical versus basal and central versus peripheral) and concomitant findings, such as nodules, cavities, and/or lymphadenopathy, that can assist in narrowing the differential diagnosis. Upper lobe volume loss has worsened, with upward hilar retraction; overall lung volumes were not decreased because of the extensive airtrapping. Despite progress in the treatment of symptoms and prevention of acute exacerbations, few advances have been made to ameliorate disease progression or affect mortality. Primary Ciliary Dyskinesia.—Primary ciliary dyskinesia, an autosomal recessive disease also known as immotile cilia syndrome, is a genetic abnormality of the dynein arms of epithelial cilia (45). Bronchiectasis is the common response of bronchi to a combination of inflammation and obstruction/impaired clearance. (b) Chest radiograph obtained 5 years later shows that bronchiectasis has progressed, with parallel linear opacities extending along the central upper lobes, representing the tram-track appearance of bronchiectasis. (a) Axial CT image shows extensive ground-glass opacity in the lower lungs, with cylindrical and varicoid bronchiectasis and bronchial wall thickening. (a) Chest radiograph obtained during evaluation of a brain mass shows an asymmetric right upper lobe opacity. Bronchiolitis Obliterans.—Bronchiolitis obliterans is clinically defined by a decrease in forced expiratory volume in 1 second by more than 20% in the presence of airflow obstruction at pulmonary function testing (54). A preexisting cartilage or motility disorder can predispose to this process. At chest radiography, a markedly dilated trachea and central bronchi can be seen (Fig 15). The number of adults with cystic fibrosis has increased over the past few decades; in 1986, only 29.2% of patients with cystic fibrosis were adults, and in 2012, almost half the patients were older than 18 years. (a) Posteroanterior chest radiograph shows upper lobe–predominant reticulonodular opacities and elevation of both hila due to upper lobe volume loss. Increased mucus secretion, decreased mucociliary clearance, airway wall thickening, and transient collapse of weakened dilated airways may contribute to the chronic obstruction that characterizes bronchiectasis; enlarged subepithelial lymph follicles or peribronchial and hilar lymph nodes with resulting bronchial narrowing may also contribute to the obstruction (1,18). As compared with honeycombing, which may present as multiple layers of cysts stacked upon one another, emphysema presents as a single layer of holes without stacking. Figure 6c Allergic bronchopulmonary aspergillosis in a 44-year-old woman with chronic cough, sinusitis, and allergic bronchopulmonary aspergillosis. 129, No. The treatment plan is highly variable, depending on several factors. North Am. In cases where bronchiectasis is severe and significant morbidity is present, surgical resection of the affected lobe may be of the benefit provided adequate respiratory reserve exists. (b) Axial CT image shows extensive cystic bronchiectasis, pronounced bronchial wall thickening with areas of mucoid bronchial impaction, and a moderate mosaic attenuation pattern indicating areas of airtrapping due to small-airways disease. Figure 4d Cystic fibrosis in a man from ages 19 to 26 years. Imaging plays a pivotal role in the diagnosis of bronchiectasis. Histologically correlating with chronic rejection after lung transplantation, bronchiolitis obliterans is a major complication in long-term follow-up of patients after lung or heart-lung transplantation, affecting 56% of patients by 3 years after transplantation (55). In the context of sarcoidosis, severe bronchiectasis is most commonly associated with the end-stage fibrosis characterizing Scadding stage IV disease. Bronchiectasis is a predominant imaging finding in cystic fibrosis, with important clinical implications. This may be due to respiratory infections, chronic granulomatous diseases, medications, and connective tissue disorders. 37, No. 2009;47 (2): 289-306. Volumetric thin-section CT can be reformatted in any projection, allowing accurate depiction of spatial distribution of abnormalities; postprocessing with minimum intensity projections can aid in detection of bronchiectasis by highlighting air-filled structures and elucidating airway course and morphologic features (22). The dependent portions of the lungs are thought to be protected from barotrauma by the typical gravitational gradient of consolidation in acute respiratory distress syndrome (40). The presence or absence of pleural effusion, thoracic lymphadenopathy (as defined by lymph node size of ≥ 10 mm in short-axis dimension), airway abnormalities (ie, airway wall thickening, bronchiectasis, and endoluminal secretions), and any underlying lung disease, including emphysema or fibrosis was also recorded. Bronchiectasis may occur as a result of various pathologic processes and thus may be a feature of a number of different lung and airway diseases (1,… Children with infectious bronchiolitis at a young age either recover completely or recover with fibrosis, which affects alveolar maturation and results in fewer alveoli and pulmonary vessels overall (52). The latter is, … In these congenital disorders, recurrent infection is also thought to play a large role in the development of bronchiectasis. In the same series, perilymphatic nodularity was identified as a ubiquitous finding at thin-section CT (31). Differences Between Bronchiectasis and COPD . Due to their multi-dimensional character and simplicity, these scores are … In addition, enlarged hilar or peribronchial lymph nodes may cause relative bronchial obstruction in chronic infection and may lead to bronchiectasis (1). 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