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Global impact of bronchiectasis and cystic fibrosis

Margarida Redondo, Holly Keyt, Raja Dhar, James D. Chalmers
Breathe 2016 12: 222-235; DOI: 10.1183/20734735.007516
Margarida Redondo
1Pulmonology Dept, Centro Hospitalar de Sao Joao, Porto, Portugal
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Holly Keyt
2University of Texas Health Science Center at San Antonio, San Antonio, TX, USA
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Raja Dhar
3Fortis Hospital, Kolkata, West Bengal, India
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James D. Chalmers
4Scottish Centre for Respiratory Research, University of Dundee, Ninewells Hospital and Medical School, Dundee, UK
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  • For correspondence: jchalmers@dundee.ac.uk
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  • Figure 1
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    Figure 1

    Radiological heterogeneity of bronchiectasis. Two patients are shown: a) a patient with moderate idiopathic bronchiectasis demonstrating predominantly cylindrical dilatation, and b) a patient with severe disease and Pseudomonas aeruginosa colonisation demonstrating widespread varicose and cystic bronchial dilatation.

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    Figure 2

    High-resolution CT scanning in a 27 year old patient with homozygous ΔF508 CF. Bilateral upper lobe bronchiectasis is evident, with mosaicism. The scan also showed extensive emphysema.

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    Figure 3

    Two images from the same individual with tracheobronchomegaly showing a) gross tracheal dilatation and b) severe central bronchiectasis.

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    Figure 4

    Venn diagram illustrating the overlap between COPD, asthma and bronchiectasis.

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  • Table 1

    Selected causes of bronchiectasis with their reported prevalence

    Aetiology of bronchiectasisProportion of adult patients affectedTypical clinical features
    Idiopathic20–60%Variable presentation
    Post-infective20–40%History of severe infection in the affected lobe(s) or historical infection#
    Post-TB<2% in developed countries
    Dominant cause in Eastern Europe, Asia and possibly other regions
    History of TB with cavitation and lung damage
    ABPA1–10%History of asthma, sputum plugs, wheezing, response to corticosteroids and demonstration of specific allergy to Aspergillus
    Central bronchiectasis
    CTD1–10%Evidence of systemic CTD (most frequently rheumatoid arthritis)
    Immunodeficiency<5%May be evidence of nonpulmonary infections or unusual microorganisms
    CF<1% in “non-CF” adult clinicsUpper lobe bronchiectasis, extrapulmonary features, male infertility, malabsorption
    Isolation of P. aeruginosa, S. aureus or NTM
    PCD<2% but may be underestimated due to limited testingMiddle or lower lobe disease, history of otitis media and rhinosinusitis, early age of onset
    Yellow nail syndrome<1%Yellow nails, pleural effusion
    Tracheobronchomegaly<1%Characteristic radiological appearance of tracheal dilatation and central bronchiectasis
    NTM infection2–50%Middle lobe nodular bronchiectatic disease
    More common in females
    May be scoliosis, pectus excavatum and low BMI
    HIV infection<1%No characteristic phenotype
    Haematological malignancy<1%Frequent respiratory tract infections
    AATD<1%Paraseptal emphysema, airflow obstruction
    Inhaled foreign body<1%Single lobe disease
    COPD¶2–60%Mild bilateral lower lobe disease with empysema and history of cigarette smoking
    Asthma¶1–50%Not well described
    Gastro-oesophageal reflux or aspiration¶N/ABilateral lower lobe or isolated right lower lobe bronchiectasis
    Not clearly identified as an aetiology
    • BMI: body mass index; N/A: not available. #: reverse causation and recall bias make these estimates unreliable; ¶: whether these are true causes of bronchiectasis is debated. Prevalence rate data were extracted from [1, 12, 15–19].

  • Table 2

    Bradford–Hill criteria for causality applied to the association between COPD, asthma and bronchiectasis

    Bradford Hill criteriaCOPDAsthma
    Strength of association/effect sizeSome studies report strong associations but may have methodological limitationsStrong association demonstrated
    Consistency/reproducibilityReported by several studies with most reporting a high prevalenceReported by several studies with most reporting a high prevalence
    SpecificityLacking, as there are often other possible causes presentLacking, as there are often other possible causes present
    TemporalityRarely demonstratedRarely demonstrated
    Biological gradientClearly demonstrated across multiple studies that as COPD becomes more severe bronchiectasis is more prevalentSome evidence that bronchiectasis is more common in more severe asthma
    PlausibilityBoth COPD and bronchiectasis are characterised by neutrophilic inflammation and share common features and therefore probably some common pathophysiologyStrong association between bronchiectasis and ABPA proves an association with eosinophilic inflammation, therefore association with asthma is highly plausible
    CoherenceNot fully testableNot fully testable
    ExperimentExperimental models of COPD are generally poor and conclusions cannot be drawnExperimental models of asthma do not show features of bronchiectasis
    AnalogyCOPD fits the model of the vicious cycle of bronchiectasis, being characterised by airway inflammation, infection and airway damageAsthma fits the model of the vicious cycle of bronchiectasis, being characterised by airway inflammation, infection and airway damage

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    • J.D. Chalmers
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Vol 12 Issue 3 Table of Contents
Breathe: 12 (3)
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Global impact of bronchiectasis and cystic fibrosis
Margarida Redondo, Holly Keyt, Raja Dhar, James D. Chalmers
Breathe Sep 2016, 12 (3) 222-235; DOI: 10.1183/20734735.007516

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Global impact of bronchiectasis and cystic fibrosis
Margarida Redondo, Holly Keyt, Raja Dhar, James D. Chalmers
Breathe Sep 2016, 12 (3) 222-235; DOI: 10.1183/20734735.007516
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  • Article
    • Abstract
    • Abstract
    • What is bronchiectasis?
    • What causes bronchiectasis?
    • What are the links with “traditional” airway diseases (COPD and asthma)?
    • What is the clinical presentation of bronchiectasis?
    • Clinical impact of bronchiectasis
    • Clinical impact of CF
    • Bronchiectasis and CF in Europe
    • Bronchiectasis and CF in North America
    • Bronchiectasis and CF in Asia
    • Management
    • Future directions
    • Conclusion
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  • Management of difficult-to-treat asthma in adolescence and young adults
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