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Bronchiectasis: a case-based approach to investigation and management

Martina Contarini, Simon Finch, James D. Chalmers
European Respiratory Review 2018 27: 180016; DOI: 10.1183/16000617.0016-2018
Martina Contarini
1Dept of Pathophysiology and Transplantation, University of Milan, Milan, Italy
2Internal Medicine Dept, Respiratory unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
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Simon Finch
3Division of Molecular and Clinical Medicine, University of Dundee, Ninewells Hospital and Medical School, Dundee, UK
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James D. Chalmers
3Division of Molecular and Clinical Medicine, University of Dundee, Ninewells Hospital and Medical School, Dundee, UK
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  • FIGURE 1
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    FIGURE 1

    Varicose and cystic bronchiectasis with mucus plugging in upper lobes.

  • FIGURE 2
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    FIGURE 2

    Central tubular bronchiectasis in upper lobes.

  • FIGURE 3
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    FIGURE 3

    a) Chest radiograph showing dextrocardia. b) High-resolution computed tomography showing cylindrical bronchiectasis in the lingula.

  • FIGURE 4
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    FIGURE 4

    Cylindrical bronchiectasis and tree-in-bud pattern in lower lobes and middle lobe.

  • FIGURE 5
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    FIGURE 5

    Different radiological “phenotypes” of bronchiectasis. a) Tubular bronchiectasis in lower lobes, b) varicose bronchiectasis in lower lobes, c) cystic bronchiectasis in right lower lobe, d) cystic bronchiectasis with tree-in-bud pattern and mucus plugging in lower lobes (cavitation in right lower lobe), e) mucus plugging and tree-in-bud pattern in lower lobes, f) varicose bronchiectasis in upper lobes and lingula.

Tables

  • Figures
  • TABLE 1

    Aetiologies of bronchiectasis

    CategoryCause/notesClinical phenotypeSpecific treatment
    Post-infectionViral, bacterial, fungal, mycobacteria (usually classified separately)Past history of severe infection; classically unilobar bronchiectasisNo specific treatment
    NTMM. avium and M. abscessus most frequentMiddle-aged or elderly; females with low BMI; middle lobe and lingual nodular bronchiectasis; cavitation; tree-in-budAntibiotic treatment
    Post-TBM. tuberculosisUpper lobe most frequentlyNo specific therapy
    ABPAHypersensitivity to A. fumigatusHistory of asthma (not universal); thick sputum; S. aureus in sputum; central bronchiectasis; fleeting infiltratesSteroids±antifungals
    COPDSmoking, biomass exposureFixed airflow obstruction; smoking history; bilateral lower lobe; tubular bronchiectasisNo specific therapy
    AsthmaNot universally accepted as a cause of bronchiectasisLong history of asthma; frequent exacerbations; neutrophilic airway inflammationInhaled corticosteroids, biologics e.g. anti-IgE and anti-IL5
    Aspiration/inhalationForeign body aspiration, gastric contents aspiration, inhalation of corrosive substancesLower lobe bronchiectasisSpeech and language therapy, fundoplication, removal of exacerbating drugs
    ObstructionBenign tumours, enlarged lymph nodesSingle lobe bronchiectasisRemoval of obstruction via bronchoscopy or thoracic surgery
    Congenital defects of large airwaysMarfan syndrome, Mounier-Kuhn syndrome (tracheobronchomegaly), Williams–Campbell syndrome (bronchial cartilage deficiency)Specific features depending on the congenital defectDependant on the underlying disorder
    AATDUnopposed protease activityCombined emphysema and bronchiectasisAugmentation therapy is available in some countries
    Yellow nail syndromeLymphatic obstructionDystrophic nails, pleural effusions, rhinosinusitisLocal treatment for nails e.g. vitamin-E, management of lymphoedema
    Immunological defectsPrimary: common variable immune deficiency, agammaglobulinemia, hyper-IgE syndrome; secondary: chemotherapy, immunosuppressant therapy, malignancy, HIV/AIDSVaried clinical pattern depending on the underlying cause; patient may give a history of non-respiratory infectionsIg replacement, prophylactic antibiotics, treatment of underlying disorder, removal of iatrogenic immunosuppression
    Young's syndromeCause not knownBronchiectasis, rhinosinusitis and reduced fertilitySee ciliary disorders below
    PCDGeneticMiddle lobe and lower lobe bronchiectasis; rhinosinusitis; middle ear infections; situs inversus in some casesRecognition and treatment of associated problems (including rhinosinusitis, middle ear disease, infertility, ectopic pregnancy), genetic counselling, intensive airway clearance
    Systemic inflammatory diseaseRheumatoid arthritis, sarcoidosis, systemic lupus erythematosus, Sjögren syndromeVaried clinical pattern, often rapidly progressiveNo specific treatment
    Inflammatory bowel diseaseUlcerative colitis, Crohn's syndrome, coeliac diseaseVaried clinical pattern often high sputum volumes and steroid responsiveInhaled and systematic corticosteroids, treatment of the underlying condition
    Adult CFCFTR mutationsUpper lobe bronchiectasis; P. aeruginosa or S. aureus in sputum; non-respiratory manifestationsSpecialist multidisciplinary care in adult CF centres, recognition and treatment of non-respiratory manifestations, CFTR modulator/corrector therapy
    Diffuse panbronchiolitisIdiopathic inflammatory diseaseMostly patients of Far Eastern ethnic originMacrolide antibiotics

    NTM: nontuberculous mycobacteria; TB: tuberculosis; ABPA: allergic bronchopulmonary aspergillosis; COPD: chronic obstructive pulmonary disease; AATD: α1-antitrypsin deficiency; PCD: primary ciliary dyskinesia; CF: cystic fibrosis; M. avium: Mycobacterium avium; M. abscessus: Mycobacterium abscessus; M. tuberculosis: Mycobacterium tuberculosis; A. fumigatus: Aspergillus fumigatus; S. aureus: Staphylococcus aureus; BMI: body mass index; Ig: immunoglobulin; IL: interleukin; CFTR: cystic fibrosis transmembrane conductance regulator; P. aeruginosa: Pseudomonas aeruginosa.

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    Bronchiectasis: a case-based approach to investigation and management
    Martina Contarini, Simon Finch, James D. Chalmers
    European Respiratory Review Sep 2018, 27 (149) 180016; DOI: 10.1183/16000617.0016-2018

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    Bronchiectasis: a case-based approach to investigation and management
    Martina Contarini, Simon Finch, James D. Chalmers
    European Respiratory Review Sep 2018, 27 (149) 180016; DOI: 10.1183/16000617.0016-2018
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