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Pictorial essay in bronchiectasis: diagnosis and beyond

Eseu pictorial în bronşiectazii: diagnostic și nu numai

Oxana Munteanu1, Dumitru Chesov1,3,

Leonid Onea2, Victor Botnaru1

1. “Nicolae Testemițanu” State University of Medicine and Pharmacy, Department of

Internal Medicine, Division of Pneumology and Allergology,

Chișinău, Republic of Moldova

2. German Diagnostic Center, Chișinău, Republic of Moldova

3. Research Center Borstel, Germany

Corresponding author: Oxana Munteanu,

E-mail: oxana.munteanu@usmf.md

Progresele în tehnologia imagistică au îmbunătățit rata de detectare a bronșiectaziilor, fapt conturat și de interesul continuu din ultimii ani pentru această maladie, inclusă anterior în grupul celor orfane. Accesibilitatea metodelor de diagnostic imagistic a amplificat performanțele diagnostice, atât prin identificarea unor indicii pentru diagnosticul etiologic al bronșiectaziilor, cât și prin evidențierea unor scoruri imagistice cu un potențial predictiv pentru evoluția acestora. Review-ul prezintă un rezumat al indiciilor diagnostice oferite de arsenalul instrumentelor imagistice disponibile și o introducere a scorurilor imagistice propuse în prezent pentru practica clinică și de cercetare la pacienții cu bronșiectazii.Cuvinte-cheie: etiologia bronşiectaziilor, HRCT, scoruri imagistice

Abstract Rezumat

Progress in imaging technology has improved the diagnostic performance in bronchiectasis, which is outlined by the continuing interest in recent years for this disease, previously considered as an orphan disease. The accessibility of diagnostic imaging tools has enhanced the diagnostic performance, both by identifying clues for the etiological diagnosis of bronchiectasis, as well as by highlighting imaging scores with a potential predictive role. The purpose of this review is to summarize diagnostic leads provided by main available imaging tools and to introduce imaging scores currently available for clinical practice and research in bronchiectasis patients.Keywords: bronchiectasis etiology, HRCT, imaging scores

IntroductionBronchiectasis has been a neglected disease for dec-

ades. However, in the past years, a continuous interest for this area of respiratory medicine has been observed. This is partly due to a larger availability of noninvasive imaging tools required for bronchiectasis diagnosis. Imaging plays an essential role in the assessment of disease severity and the detection of bronchiectasis complications; it could even suggest the bronchiectasis etiology. Nevertheless, the role of imaging assessment for disease prognosis and follow-up is not clear yet. A significant input in this regard could be provided by bronchiectasis imaging scores; how-ever, their applicability in routine clinical practice has not been well established yet. The purpose of the review is to summarize diagnostic opportunities provided by main available imaging tools and to describe imaging scores currently proposed for clinical practice and research in patients with bronchiectasis. The images were sourced from the Pneumology/Allergology Clinic of the “Nicolae Testemiţanu” State University of Medicine and Pharmacy, Chişinău, Republic of Moldova. The patients’ consent was obtained.

Imaging for bronchiectasis diagnosisCHEST RADIOGRAPHYChest radiography is commonnly used as a first

intention diagnostic tool for bronchiectasis. However, it misses sensitivity and does not detect mild or even moderate disease. In patients with chronic sputum pro-duction and bronchographically proven bronchiectasis, the overall sensitivity of chest radiography does not exceed 50%(1). A normal chest radiograph or one with nonspecific lesions is a common finding. So far, plain

chest radiography is enough accurate only for the assess-ment of advanced lesions. That’s why the role of radiog-raphy in clinical management of bronchiectasis is currently reduced to ruling out some complications as intercurrent infections, progressive lobar collapse or cavitary disease in a patient with known disease.

Several radiological signs associated with bronchi-ectasis could be conventionally divided in direct and indirect ones.

Direct signsTram track sign is characterized by near-parallel lin-

ear opacities, resembling a railway, reflecting luminal dilatation and variable wall thickening (Figure 1A).

Signet ring sign mirrors bronchial dilatation when the major axis of bronchiectasis runs parallel to the radia-tion beam. Signet ring and tram track signs essentially reflect the same anatomic abnormality but in two dif-ferent projections. In case of plentiful bronchial secre-tions, an air-fluid level could be seen within the ring (Figure 2A). Tubular opacities reflect the mucus filled bronchi.

Indirect signsLung volume increasement is associated with bronchi-

ectasis in patients with obstructive pulmonary disease (Figure 2B).

Asymmetric volume reduction with parenchymal opaci‑fication is due to segmental or lobar atelectasis and is accompanied by fissure displacement and/or obscura-tion of the diaphragm (Figure 2A).

Vascular lung structures may be more expressed due to fuzzy outlines caused by contiguous peribronchial inflammatory infiltrates and chronic fibrotic changes (Figure 3 A, B).

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Figure 1. Posteroanterior chest X-ray (A) in a 32-year-old patient, with idiopathic bronchiectasis diagnosed during childhood, shows a subtle tram track (arrows) appearance in the lower fields representing bronchiectasis and bronchial wall thickening. High-resolution computed tomography shows widespread cylindrical bilateral bronchiectasis predominantly in lower lung fields, nontapering bronchi (C; white arrows) and mucus plugging (B, D; black arrows).

Figure 2. A – Posteroanterior chest radiograph in a 37-year-old patient showing bilateral gross cystic bronchiectasis mostly at the lung bases, presenting as a group of multiple cystic airspaces with obscuration of the diaphragm. Note the presence of air-fluid levels (arrows) within the rings. Air-fluid levels may be present in bronchiectasis and are usually not seen in patients with lung cysts. B – Posteroanterior chest radiograph showing overinflated lungs and bilateral cystic bronchiectasis in the lower lung fields.

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COMPUTED TOMOGRAPHYThoracic computed tomography (CT) is more sensitive

than radiography for bronchiectasis imaging. CT is consid-ered the gold standard for the assessment of the extension and type of structural changes of both bronchi and lung parenchyma. It allows to describe morphological types of bronchiectasis (cylindric, varicose, or cystic), many patients having a combination of the classical types described by Reid(2). The most common direct bronchiectasis signs revealed by CT are: increased broncho-arterial ratio (signet ring sign – Figures 1D and 4A), parallel linear opacities (tram track sign), lack of bronchial tapering, bronchi visible within 1 cm of subpleural parenchyma. All aforementioned CT signs are unspecific for bronchiectasis etiology. Small cylin-

drical bronchiectasis in a single pulmonary segment appears in a significant number of healthy adults; therefore, it should not be considered(3,4).

Other CT lesions associated in bronchiectasis are mucus plugging opacities, cysts, bullae and air trapping (caused by a vicious cycle of infection, inflammation and mucus deposition).

Mucus plugging is easily identified as complete or partial luminal filling (Figures 1B, 1D, 3A). Secondary changes in the adjacent lung parenchyma include volume loss, mosaic attenu-ation due to air trapping, fibrosis, and scarring. Collapsed lung areas secondary due to mucus plugging (Figure 5) can be iden-tified in more advanced and long-standing disease(5). The detection of patchy consolidations of adjacent lung paren-

Figure 3. Chest radiographs of a 62-year-old patient with idiopathic bronchiectasis (diagnosed 16 years ago) before (A) and after antibiotic treatment (B) for infectious exacerbation. There are over-inflation of the lungs, exten-sive bilateral bronchiectasis with peribronchial thickening and patchy consolidation seen on posteroanterior chest radiograph (A). After the antibacterial treatment (B) the ring lines are thinner and there are clear borders of the heart and diaphragm. An axial (C, D) and coronal (E) CT scan with parenchymal lung window settings (performed after exacerbation treatment) shows diffuse bronchiectasis; all morphological types could be seen.

Figure 4. Basic morphologic types of bronchiectasis recognized on CT. Axial CT images show bronchial dilatation with mild bronchial wall thickening characteristic for cylindrical bronchiectasis, which is the most common morphological type (A), varicoid bronchiectasis (B – characterized by an undulating irregular contour with luminal enlargement and interposed stenosis) and cystic bronchiectasis (C, D) that appears as saccular dilatation of the airways and air-fluid levels from mucus stagnation.

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Figure 5. High-resolution computed tomography images showing collapsed left lower lobe with multiple cystic bronchiectasis in a 76-year-old woman diagnosed with bronchiectasis after a haemoptysis episode. Lymph node calcification (arrow) could suggest a postinfectious etiology of bronchiectasis.

Etiology of bronchiectasis Findings DistributionAllergic bronchopulmonary

aspergillosis High-attenuation mucus plugging, seen as “finger in glove” sign Upper lung zone, central

Tuberculosis Asymmetric upper lobe involvement, tree-in-bud nodules Upper lung zone, focal or unilateral

Chronic atypical mycobacterial infection

Most common in males with chronic obstructive pulmonary disease or in thin, older women; indolent symptoms Middle lobe and lingular

Swyer-James (McLeod) syndrome Bronchial wall thickening, unilateral hyperlucency due to air trapping Focal or unilateral

Cystic fibrosis Extensive cystic and cylindrical bronchiectasis Upper lung zone, centralPrimary ciliary dyskinesia Situs inversus, chronic sinusitis, and bronchiectasis Middle lobe and lingularMounier-Kuhn syndrome Excessive dilatation of the trachea and main bronchi Central

Williams-Campbell syndrome Cartilage deficiency of the midorder bronchi Midorder bronchi

Foreign bodies Foreign body may be of soft tissue or high density lesions and lack any enhancement.Organic foreign body may show internal air foci due to entrapped air Focal or unilateral

Recurrent aspiration Bronchial wall thickening, aspirated material in bronchi, presence of hiatal hernia Lower lung zone,peripheral

Endobronchial tumor Extraluminal invasion and lymphadenopathy are corroborative findings to suggest a neoplasm. Volume loss secondary to endobronchial lesions in main or segmental bronchi Focal or unilateral

Etiology of bronchiectasis and suggestive HRCT findingsTable 1

chyma (Figure 3A), a more expressed peribronchial thickening, the presence of air-fluid levels, or centrilobular nodules are suggestive for superimposed infection.

Despite its high sensitivity, even CT imaging sometimes does not provide straightforward answers regarding bron-chiectasis diagnosis. So far, cystic bronchiectasis could appear as a group of clustered air-filled “cysts-like” struc-tures that require differential diagnosis with true paren-chymal cysts. The presence of accompanying pulmonary artery branch (the signet ring sign) or scrolling through the adjacent scan slices figuring out the tubular nature of the “cyst-like” structure allow the differentiation between two entities (Figure 4C, D).

Additionally, the eventual presence of the air-fluid levels and patchy distribution argue in favor of bronchiectasis. Cystic lung diseases (lymphangiomyomatosis or Langerhans histiocytosis) often have a bilateral diffuse spreading.

Volumetric and multiplanar reconstruction could be helpful in the differential diagnosis between bronchiectasis and a range of cystic abnormalities, in particular honeycombing.

CT scan is not recommended for bronchiectasis fol-low-up, especially in case of young and female patients. That is mainly due to significant radiation risk without any clinical benefits(4,6).

IMAGING CLUES TOWARDS ETIOLOGYThe wide list of differential diagnosis in bronchiectasis

can be substantially narrowed by considering their ana-tomic location and distribution(7). Some CT features of bronchiectasis may provide important clues regarding the etiology (Table 1)(8-10). Thus, severe bronchiectases are often seen in the context of congenital disorders such as cystic fibrosis, congenital immunodeficiency, or abnor-malities of cartilage development (Figures 6-9).

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The predominant involvement of the subsegmental bronchial branches is suggestive for Williams-Campbell syndrome (Figure 6) – a rare form of congenital bronchi-ectasis characterized by deficiency of cartilage in subseg-mental bronchi, usually diagnosed in childhood or sometimes in adult age(11).

Marked dilatation of the trachea and main bronchi is definitory for Mounier-Kuhn syndrome (Figure 7) caused by primary atrophy of the musculo-elastic tissue. With a

higher incidence in males, idiopathic tracheobroncho-megaly is usually diagnosed in the 3rd or 4th decade of life. The clinical presentation varies from minimal disease with well-preserved pulmonary function to severe bron-chiectasis with emphysema and pulmonary fibrosis lead-ing to respiratory failure and death.

Bronchiectasis in cystic fibrosis (CF) usually has a bilateral, proximal, perihilar and upper lobe predomi-nance. CT images show extensive cystic and cylindrical

Figure 6. Williams-Campbell syndrome diagnosed in a 56-year-old woman, known with recurrent lower respiratory tract infections and chronic cough since childhood. Posteroanterior chest radiograph (A) demonstrating sparse reticular opacities more evident in the lower lobes, besides bronchiectasis. Bronchiectases are more obvious seen in the high-resolution computed tomography images (B-E) that show predominantly varicose and cystic central bronchiectasis in all lobes, limited to the mid-order subsegmental airways. Note the thin-walled cystic bronchiectasis and normal calibre of the trachea and proximal bronchi. The patient died of respiratory failure one year later.

Figure 7. Mounier-Kuhn’s syndrome in a 47-year-old patient, ex-smoker, 10 pack-years. Chest radiograph (A) and HRCT lung window coronal images (B, C) showing marked tracheal enlargement (arrows) involving grossly dilated mainstem and lobar bronchi (arrows). Note the corrugated contour of the tracheal wall, bilateral branching thin-walled cystic lesions located centrally in the lung, consistent with bronchiectasis, and bullous emphysema in the right upper lobe.

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bronchiectasis (typically, more extensive than in non-CF cases), thick bronchial wall and peribronchial intersti-tial thickening (Figure 8). Upper lobe predominance is commonly seen, but it is not mandatory. A mosaic pat-tern of attenuation secondary to air-trapping, tree-in-bud nodules and diffuse distribution of bronchiectasis are also common findings(9).

Due to genetically determined abnormal chloride transportation, CF bronchiectases are associated with reproductive tract and digestive system abnormalities. The classic diagnostic triad in patients with CF includes an abnormal sweat chloride test and signs of pulmonary and pancreatic disease.

A finding of predominantly middle and basal distrib-uted bronchiectasis is suggestive for primary ciliary dys-kinesia (PCD), also known as immotile cilia syndrome(12,13). It is a genetic abnormality of the dynein arms of epithelial cilia that causes an impaired muco-ciliary clearance, resulting in chronic oto-sino-pulmonary disease. Bronchiectasis is a consistent finding in all adults with PCD. A subset of PCD patients could present with a triad of bronchiectasis, situs inversus, and either chronic sinusi-tis, or nasal polyps, which is known as Kartagener syn-

drome (Figure 9). PCD is considered the most common congenital cause for non-CF fibrosis bronchiectasis.

Bilateral centrally distributed bronchiectasis is a com-mon feature of bronchial lesions in patients with allergic bronchopulmonary aspergillosis (ABPA). About 75-95% of ABPA patients have CT identifiable bronchiectasis(14,15). High-attenuating (>70 to 100 HU) bronchial content is reported in 18-28% of patients with ABPA (Figure 10). That usually represents fungal debris containing iron and man-ganese and is considered a characteristic ABPA feature(16,17). Bronchiectasis in ABPA is a consequence of chronic airway inflammation, damage and remodeling resulting from a reaction to the presence of endobronchial Aspergillus species in asthma and cystic fibrosis patients. The diagnostic cri-teria for ABPA include symptoms of bronchial asthma, immediate skin test reactivity to Aspergillus fumigatus, elevated serum immunoglobulin E levels, pulmonary infil-trates, central bronchiectasis, peripheral blood eosinophilia and the presence of serum precipitins against Aspergillus antigen(9,18). ABPA should be always suspected in patients with recurrent asthma exacerbations, expectoration of dark mucus plugs, hemoptysis and/or systemic symptoms such as fever and malaise.

Figure 8. Chest radiographs in a 25-year-old woman with cystic fibrosis, showing severe cystic bronchiectasis predominant on the right upper field with decreased right lung volume, important upper lobe volume loss from collapsed lobe with upward hilar retraction, pleural thickening and mediastinal traction to the right. Reticular opacities throughout the left lung, expressing bronchial wall thickening and nodular/tubular opacities representing mucoid bronchial impaction (A, B). Computed tomography proved the severe lesions on the right: tubular, varicose and cystic bronchiectasis with bilateral severe emphysema. Note several large cavities with thick walls in the right upper lobe and one cavity in the left upper lobe with adjacent consolidation (C-F).

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Figure 9. Kartagener syndrome in a 31-year-old woman, diagnosed with PCD in the first year of life and with bronchiectasis at 6 years old. Posteroanterior chest radiograph (A) demonstrates situs inversus totalis, with dextrocardia and the stomach in the right upper quadrant of the abdomen. HRCT images (B, C – coronal; D – sagittal; E - axial) show widespread bilateral bronchiectasis with a predilection for the central part, cystic bronchiectasis in the middle lobe, lingula and right lower lobe. Mucoid impaction (C – arrows) with “finger in glove” sign seen in the right upper lobe and patchy areas of tree-in-bud nodules (small airways dysfunction) seen in the left lower lobe characterise the infectious exacerbation of bronchiectasis. Occipitomental X-ray examination (the Waters view) demonstrated opacified paranasal sinuses (F). 

Bronchiectasis of apical and posterior upper lobe segments is highly suggestive for tuberculous origin, particularly in patients from high TB burden countries. The bronchiectasis could be the expression of post-pri-mary active tuberculosis (30-60% of TB patients), as well as scarring due to prior infection (71-86% of TB cured patients)(19). Post-tuberculous bronchiectasis is rarely gross, usually asymmetric with upper lobe pre-dominance. In previously treated TB patients bronchi-ectasis can remain stable for years. Lung volume loss, calcified lymph nodes and parenchymal calcifications are frequently found as accompanying features (Figures 11 and 12). Tree-in-bud pattern and cavities could sug-gest the reactivation of TB infection.

Focal bronchiectasis is usually caused by airway obstruction by a variety of causes. The differential diag-nosis includes tumors, endobronchial foreign body, extrinsic compression or post-infectious broncholithi-asis. Bronchial compression could be caused by lymphad-enopathy usual ly from previous granulomatous

infection, sarcoidosis, hilar mass or metastatic lymphad-enopathy. Broncholithiasis describes calcified material within the bronchial lumen secondary to granulomatous infections such as tuberculosis or non-tuberculous mycobacteria. Diagnostic work-up in bronchiectasis sec-ondar y to air way obstruction usual ly implies bronchoscopy(7,10,20).

Beside the site of bronchus obstruction, patient’s age may be helpful in predicting the bronchiectasis origin. Intraluminal occlusion in adults is more frequent due to neoplasms, of which carcinoid is the commonest cause of long-standing occlusion. Foreign body is a typical intra-luminal cause of bronchiectasis in children. In the major-ity of the cases, foreign body aspiration involves the right mainstem bronchus followed by left mainstem bronchus due to their alignment in line with the trachea. In patients without acute symptoms, the aspirated foreign bodies may remain undetected for months or even years and are often misdiagnosed (Figure 13). Undiagnosed and retained aspi-rated foreign bodies may cause recurrent infections, lead-

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Figure 11. Destruction of the right middle lobe from previous tuberculosis in a 71-year-old woman, treated at the age of 24 with right upper lobectomy. Frontal and lateral view radiographs (A, B) show severe fibrotic changes with bronchiectasis on the right, with lung volume loss and marked shifting of the mediastinum toward the right, several tubular hyperlucencies inside the right middle lobe opacity caused by traction bronchiectasis (arrows), a lot of parenchymal calcifications bilaterally, and hyperinflation of the left lung due to compensatory emphysema. HRCT scan shows the right middle lobe entirely destroyed with varicose and cystic bronchiectasis, and the calcification of bronchial walls (C). Note that both the right and left heart borders are obscured (A), the presence of calcified lymph node (E) in the pretracheal area (arrows), and bronchiectasis with fibrosis in the left lower lobe (F, G).

Figure 10. Allergic bronchopulmonary aspergillosis in a 49-year-old woman with a 23-year history of bronchial asthma. Chest radiographs (A, B) show ectatic bronchi with mucoid impaction and the classic finger in glove sign in the upper lobes. Coronal HRCT (C – mediastinal window) showing high-attenuating mucoid impaction of upper lobes bronchiectasis (arrows). High iron and manganese content of the fungal debris, explaining the high attenuation. Axial, coronal and sagittal HRCT (D, E, F – lung window) confirming the classic presentation of extensive central upper bronchiectasis (arrows), with some sparing bronchiectasis and typical signet ring sign.

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Figure 13. Occult foreign body aspiration leads to localised bronchiectasis. A 20-year-old female patient admitted for haemoptysis and signs of respiratory infection had aspirated a pen cap when she was 11 years old, but denied any serious respiratory complication at that time or later. She was admitted for the first time 6 months prior the current presentation with right lower lobe pneumonia and discharged home following parenteral antibiotic therapy. PA chest radiograph (A) shows a consolidation syndrome in the right lower field, elevation and loss of the normal right hemidiaphragm contour. HRCT images show “finger in glove” sign, the bronchus distal to the obstruction being dilated with mucus plugging. Note the bronchiectatic dilatations along with peribronchial thickening and segmental pneumonic consolidations in the right lower lobe (B-D). A picture (E) during the removal of the foreign body (a pen cap – F) using fiberoptic bronchoscope.

Figure 12. Post-tuberculous bronchiectasis in a 53-year-old woman with bronchial asthma from childhood. No history of treated tuberculosis. Posteroanterior chest radiograph (A) shows loss of the normal right medial cardiomediastinal contour, increased density in the upper medial area of the right hemithorax, a volume loss of right upper lobe with the elevation of the horizontal fissure (arrows). CT images (coronal, sagittal and axial) show the classical appearance of varicoid bronchiectasis, extending up to the periphery. The calcification of bronchial wall with small parenchymal calcinates (B) and bronchoscopic findings (black pigmentation presented as velvety irregular dark mucosa in the superior segment of the right upper lobe – E) suggestive for sequelae of the previously healed tuberculosis.

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ing to bronchiectasis. Inorganic foreign bodies are known to cause fewer secondary infections compared to those of organic origin(21).

IMAGING SCORING SYSTEMStructural lung changes seen on CT scans in bron-

chiectasis patients could be expressed by several scoring systems. These can be used in adjunction to clinical parameters to assess severity and predict disease evolu-tion(22-24). Currently proposed scores try to quantify the extension and imaging severity of bronchiectasis. For disease extension, the assessment usually considers either the number of the affected segments, or the per-centage of each lung lobe involvement(25).

The Bhalla score (Table 2), based on thin section CT appearances of CF bronchiectasis, was published in 1991(25). However, currently it is used for the assessment of bronchiectasis of any etiology(24). The Bhalla score is a quite complex one, assessing nine CT features by three categories of severity. That makes it quite laborious for routine application. The Reiff score (Table 3) is a simpler one, and similarly to the Bhalla score, it is focused on the assessment of imaging lesions extension(24). A sig-nificant limitation of both Bhalla and Reiff systems is that quantity and extension of imaging lesions do not always reflect the rate of airway damage, namely, the disease activity. So far, a patient can have a similar score either due to a severe localized disease, or as result of a

Category 1 2 3

Severity of bronchiectasisMild: luminal diameter slightly larger

than diameter of the homologous artery

Moderate: luminar diameter 2-3 times the diameter of the

homologous artery

Severe: luminar diameter >3 times the diameter of the homologous

artery

Peribronchial thickeningMild: bronchial wall thickness equal to the diameter of the homologous

artery

Moderate: bronchial wall thickness ≤2 times the diameter of the

homologous artery

Severe: bronchial wall thickness >2 times the diameter of the

homologous artery

Extent of bronchiectasis (number of involved segments) 1-5 6-9 >9

Extent of mucus plug (number of involved segments) 1-5 6-9 >9

Sacculation or abscesses (number of involved segments) 1-5 6-9 >9

Bronchial generation with bronchiectasis and/or mucus plug ≤fourth generation ≤fifth generation Diffuse bronchiectasis including

distal bronchi

Bullae: distribution and total number Unilateral ≤4 Bilateral ≤4 >4

Emphysema (number of involved segments) 1-5 >5 ND

Extent of collapse and/or consolidation Subsegmental Segmental or lobar ND

ND – Not defined; the value “0” is omitted in the table because it always reflects the absence of the aforementioned category

Radiological bronchiectasis score proposed by Bhalla et al.(4,25)

Radiological bronchiectasis score proposed by Reiff et al.(4,27)

Table 2

Table 3

Category of individual lobar involvement 1 2 3

Extent of bronchiectasis One or partial bronchopulmonary segment involvement

Two or more bronchopulmonary segment involvement ND

Severity of bronchiectasis Luminal diameter <2 times the diameter of the homologous artery

Luminal diameter 2-3 times the diameter of the homologous artery

Luminal diameter >2 times the diameter of the homologous artery

Peribronchial thickening Bronchial wall thickness 0.5 the diameter of the homologous artery

Bronchial wall thickness 0.5-1 the diameter of the homologous artery

Bronchial wall thickness >1 the diameter of the homologous artery

ND – Not defined; the value “0” is omitted in the table because it always reflects absence of the aforementioned category

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9. Milliron B, Henry TS, Veeraraghavan S, et al. Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases. Radiographics: a review publication of the Radiological Society of North America, Inc. 2015; 35(4):1011-30.

10. Singh A, Bhalla AS, Jana M. Bronchiectasis Revisited: Imaging-Based Pattern Approach to Diagnosis. Current problems in diagnostic radiology. 2018.

11. Jones QC, Wathen CG. Williams-Campbell syndrome presenting in an adult. BMJ case reports. 2012.

12. Dettmer S, Ringshausen F, Vogel-Claussen J et al. Computed tomography in adult patients with primary ciliary dyskinesia: Typical imaging findings. PloS one. 2018; 13(2):e0191457.

13. Shapiro AJ, Zariwala MA, Ferkol Tet al. Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatric pulmonology. 2016; 51(2):115-32.

14. Mitchell TA, Hamilos DL, Lynch DA et al. Distribution and severity of bronchiectasis in allergic bronchopulmonary aspergillosis (ABPA). The Journal of Asthma: official journal of the Association for the Care of Asthma.

2000; 37(1):65-72.15. Neeld DA, Goodman LR, Gurney JWet al. Computerized tomography in the

evaluation of allergic bronchopulmonary aspergillosis. The American review of respiratory disease. 1990; 142(5):1200-5.

16. Agarwal R. High attenuation mucoid impaction in allergic bronchopulmonary aspergillosis. World Journal of Radiology. 2010; 2(1):41-3.

17. Agarwal R, Gupta D, Aggarwal AN et al. Clinical significance of hyperattenuating mucoid impaction in allergic bronchopulmonary aspergillosis: an analysis of 155 patients. Chest. 2007; 132(4):1183-90.

18. Walsh TJ, Anaissie EJ, Denning DW et al. Treatment of aspergillosis: clinical practice guidelines of the Infectious Diseases Society of America. Clinical infectious diseases: an official publication of the Infectious Diseases Society of America. 2008; 46(3):327-60.

19. Kim HY, Song KS, Goo JM et al. Thoracic sequelae and complications of tuberculosis. Radiographics. 2001;21(4):839-58; discussion 59-60.

20. Araujo D, Shteinberg M, Aliberti Smet al. Standardised classification of the aetiology of bronchiectasis using an objective algorithm. The European Respiratory Journal. 2017; 50(6).

21. Alharthi BJ, Masoodi I, Almourgi MA et al. Occult foreign body in the lung mimicking bronchogenic carcinoma. BMJ case reports. 2014.

22. Bedi P, Chalmers JD, Goeminne PC et al. The BRICS (Bronchiectasis Radiologically Indexed CT Score): A Multicenter Study Score for Use in Idiopathic and Postinfective Bronchiectasis. Chest. 2017.

23. Chalmers JD, Goeminne P, Aliberti S, et al. The bronchiectasis severity index. An international derivation and validation study. American Journal of Respiratory and Critical Care Medicine. 2014; 189(5):576-85.

24. Park J, Kim S, Lee YJ et al. Factors associated with radiologic progression of non-cystic fibrosis bronchiectasis during long-term follow-up. Respirology. 2016; 21(6):1049-54.

25. Bhalla M, Turcios N, Aponte V et al. Cystic fibrosis: scoring system with thin-section CT. Radiology. 1991; 179(3):783-8.

26. Martinez-Garcia MA, de Gracia J, Vendrell Relat Met al. Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score. The European Respiratory Journal. 2014; 43(5):1357-67.

27. Reiff DB, Wells AU, Carr DH et al. CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types. American Journal of Roentgenology. 1995; 165(2):261-7.

Refe

renc

eswidespread mild disease. Additionally, the scores do not consider the diverse etiologies of bronchiectases, which could have different middle- and long-term prognosis.

An alternative simplif ied CT score is BRICS (Bronchiectasis Radiologically Indexed CT Score) based on the degree of bronchial dilatation and the number of bronchopulmonary segments with emphysema, in patients with idiopathic and post-infective bronchiec-tasis with limited smoking history. The BRICS was pro-posed to be used to follow-up patients longitudinally, being a potentially useful tool for future researches(22).

Recently, a couple of multidimensional scores which combine clinical and imaging variables have been proposed: BSI (Bronchiectasis Severity Index) and FACED (F – FEV1; A – Age; C – Chronic colonization;

E – Extension; D – Dyspnea). These scores are using several clinical parameters and counting the number of lobes affected by bronchiectasis on CT scan. They seem to be more accurate in assessing severity and prognosis of the disease(23,26).

ConclusionsBronchiectasis is an irreversible disease of various eti-

ologies. HRCT is indispensable for bronchiectasis detection, assesses its morphology, distribution and disease activity. New insights into bronchiectasis management by combin-ing clinical data, functional and imaging features are still required. Accurate multimodal tools aiming to provide a personalized approach for clinical care of bronchiectasis patients should be developed. ■

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