Bronchiectasis: Difference between revisions

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== Definition ==
+
== Background ==
   
 
* Permanent dilatation of the small airways, causing persistent microbial infection
 
* Permanent dilatation of the small airways, causing persistent microbial infection
   
== Etiology ==
+
=== Etiologies ===
   
 
* Focal
 
* Focal
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*** Chronic infection
 
*** Chronic infection
 
**** Granulomatous
 
**** Granulomatous
***** Tuberculosis
+
***** [[Tuberculosis]]
***** Histoplasmosis
+
***** [[Histoplasmosis]]
***** MAC/NTM
+
***** [[MAC]]/[[NTM]]
**** Bacterial: S. aureus, Klebsiella, Pseudomonas
+
**** Bacterial: [[Staphylococcus aureus]], [[Klebsiella]], [[Pseudomonas]]
 
**** Viral
 
**** Viral
 
*** Obstruction
 
*** Obstruction
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**** Tumour
 
**** Tumour
 
*** Right middle lobe syndrome
 
*** Right middle lobe syndrome
*** Swyer-James-MacLeod syndrome (postinfective obliterative bronchiolitis)
+
*** [[Swyer-James-MacLeod syndrome]] (postinfective obliterative bronchiolitis)
 
** Congenital
 
** Congenital
 
*** Bronchopulmonary sequestration
 
*** Bronchopulmonary sequestration
*** Williams-Campbell syndrome (cartilage deficiency)
+
*** [[Williams-Campbell syndrome]] (cartilage deficiency)
*** Mouner-Kuhn syndrome (tracheobronchmegaly)
+
*** [[Mouner-Kuhn syndrome]] (tracheobronchmegaly)
 
* Diffuse
 
* Diffuse
 
** Genetic
 
** Genetic
*** Cystic fibrosis (CF)
+
*** [[Cystic fibrosis]]
*** Primary ciliary dyskinesia/Kartagener's syndrome
+
*** [[Primary ciliary dyskinesia]]/Kartagener's syndrome
*** Alpha-1 antitrypsin deficiency
+
*** [[Alpha-1 antitrypsin deficiency]]
*** Young's syndrome
+
*** [[Young syndrome]]
** Infectious: Non-tuberculous mycobacterium/MAC
+
** Infectious: [[non-tuberculous mycobacterium]]/[[MAC]]
** Immunodeficiency: Hypogammaglobulinemia
+
** Immunodeficiency: [[hypogammaglobulinemia]]
** Inflammatory: Allergic bronchopulmonary aspergillosis (ABPA)
+
** Inflammatory: [[allergic bronchopulmonary aspergillosis]]
 
** Autoimmune or immune-mediated
 
** Autoimmune or immune-mediated
*** Rheumatoid arthritis
+
*** [[Rheumatoid arthritis]]
*** Sjögren's syndrome
+
*** [[Sjögren syndrome]]
*** Inflammatory bowel disease
+
*** [[Inflammatory bowel disease]]
** Bronchiolitis obliterans after transplant
+
** [[Bronchiolitis obliterans]] after transplant
 
** Recurrent aspiration
 
** Recurrent aspiration
 
** Near drowning
 
** Near drowning
 
** Toxic inhalation
 
** Toxic inhalation
 
** Miscellaneous
 
** Miscellaneous
*** Yellow nail syndrome
+
*** [[Yellow nail syndrome]]
 
*** Postradiation traction bronchiectasis
 
*** Postradiation traction bronchiectasis
*** IPF
+
*** [[IPF]]
 
== Epidemiology ==
 
 
== Risk Factors ==
 
   
 
== Clinical Manifestations ==
 
== Clinical Manifestations ==
 
=== History ===
 
   
 
* Chronic cough (98%)
 
* Chronic cough (98%)
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* Hemoptysis (27%)
 
* Hemoptysis (27%)
 
* Recurrent pleurisy (20%)
 
* Recurrent pleurisy (20%)
 
=== Signs & Symptoms ===
 
   
 
== Investigations ==
 
== Investigations ==
   
 
* Labs
 
* Labs
** CBC, immunoglobulins, and HIV testing to rule out immunodeficiency
+
** CBC, quantitative immunoglobulins, and HIV testing to rule out immunodeficiency
 
** Sputum culture for bacteria, mycobacteria, and fungi; consider BAL
 
** Sputum culture for bacteria, mycobacteria, and fungi; consider BAL
 
** Sweat chloride testing +/- genetic testing to rule out CF
 
** Sweat chloride testing +/- genetic testing to rule out CF
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** Bronchoscopy
 
** Bronchoscopy
 
** Swallowing assessment to rule out aspiration
 
** Swallowing assessment to rule out aspiration
  +
  +
=== CT[[CiteRef::milliron2015br]] ===
  +
{| class="wikitable"
  +
!Cause
  +
!Distribution
  +
!Findings
  +
|-
  +
! colspan="3" |Infection/Aspiration
  +
|-
  +
|Recurrent aspiration
  +
|lower lung zone, peripheral
  +
|bronchial wall thickening, aspirated material in bronchi, presence of hiatal hernia
  +
|-
  +
|[[ABPA]]
  +
|upper lung zone, central
  +
|high-attenuation mucous plugging
  +
|-
  +
|[[Tuberculosis]]
  +
|upper lung zone, focal or unilateral
  +
|asymmetric upper lobe involvement, tree-in-bud nodules
  +
|-
  +
|[[Non-tuberculous mycobacteria]]
  +
|middle lobe and lingular
  +
|more common in men with [[COPD]] or in thin, older women; indolent symptoms
  +
|-
  +
|[[Swyer-James syndrome]]
  +
|focal or unilateral
  +
|bronchial wall thickening, increased lucency in the small lung due to air trapping
  +
|-
  +
! colspan="3" |Congenital
  +
|-
  +
|[[Cystic fibrosis]]
  +
|upper lung zone, central
  +
|extensive cystic and cylindrical bronchiectasis
  +
|-
  +
|[[Primary ciliary dyskinesia]]
  +
|middle lobe and lingular
  +
|situs inversus, chronic sinusitis, and bronchiectasis
  +
|-
  +
|[[Mounier-Kuhn syndrome]]
  +
|central
  +
|absence or atrophy of the elastic fibers and smooth muscle in trachea and main bronchial wall
  +
|-
  +
|[[Williams-Campbell syndrome]]
  +
|midorder bronch
  +
|cartilage deficiency in the midorder bronchi
  +
|-
  +
|[[Bronchial atresia]]
  +
|focal or unilateral
  +
|most common location
  +
|-
  +
! colspan="3" |Fibrosing Diffuse Disease
  +
|-
  +
|[[Sarcoidosis]]
  +
|upper lung zone, central
  +
|bilateral hilar lymphadenopathy, perilymphatic nodules
  +
|-
  +
|[[Pulmonary fibrosis]]
  +
|lower lung zone, peripheral
  +
|varicoid bronchiectasis, architectural distortion, honeycombing in [[UIP]]
  +
|-
  +
|[[ARDS]]
  +
|middle lobe and lingular
  +
|varicoid bronchectasis, diffuse ground-glass opacities
  +
|-
  +
! colspan="3" |Endobronchial/Peribronchial Lesions
  +
|-
  +
|Endobronchial tumour
  +
|focal or unilateral
  +
|
  +
|-
  +
|Foreign body
  +
|focal or unilateral
  +
|
  +
|-
  +
|Stricture
  +
|focal or unilateral
  +
|
  +
|-
  +
! colspan="3" |Other
  +
|-
  +
|[[Bronchiolitis obliterans]]
  +
|diffuse
  +
|significant cause of post-transplantation morbidity and mortality; diffuse air trapping
  +
|}
   
 
== Management ==
 
== Management ==
   
=== Acute exacerbation ===
+
=== Acute Exacerbation ===
   
 
* Send sputum cultures every time
 
* Send sputum cultures every time
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* Consider prednisone if asthma or ABPA
 
* Consider prednisone if asthma or ABPA
   
=== Chronic ===
+
=== Chronic Management ===
   
 
* Chest physiotherapy it most important
 
* Chest physiotherapy it most important
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** Pulmonary rehabilitation
 
** Pulmonary rehabilitation
 
** GERD treatment
 
** GERD treatment
  +
  +
== Further Reading ==
  +
  +
* British Thoracic Society Guideline for bronchiectasis in adults. ''Thorax''. 2019;74:1-69. doi: [https://doi.org/10.1136/thoraxjnl-2018-212463 10.1136/thoraxjnl-2018-212463]
   
 
[[Category:Respirology]]
 
[[Category:Respirology]]

Latest revision as of 11:19, 22 March 2022

Background

  • Permanent dilatation of the small airways, causing persistent microbial infection

Etiologies

Clinical Manifestations

  • Chronic cough (98%)
  • Daily sputum (78%)
  • Rhinosinusitis (73%)
  • Dyspnea (62%)
  • Hemoptysis (27%)
  • Recurrent pleurisy (20%)

Investigations

  • Labs
    • CBC, quantitative immunoglobulins, and HIV testing to rule out immunodeficiency
    • Sputum culture for bacteria, mycobacteria, and fungi; consider BAL
    • Sweat chloride testing +/- genetic testing to rule out CF
    • Alpha-1 antitrypsin levels (<11 in deficiency)
    • Nasal brush/biopsy to rule out ciliary dyskinesia
  • Imaging
    • High-resolution CT
      • Signet-ring sign (airway >1.5x blood vessel) is best sign
      • Tram-tracking
  • Other
    • PFTs
    • Bronchoscopy
    • Swallowing assessment to rule out aspiration

CT1

Cause Distribution Findings
Infection/Aspiration
Recurrent aspiration lower lung zone, peripheral bronchial wall thickening, aspirated material in bronchi, presence of hiatal hernia
ABPA upper lung zone, central high-attenuation mucous plugging
Tuberculosis upper lung zone, focal or unilateral asymmetric upper lobe involvement, tree-in-bud nodules
Non-tuberculous mycobacteria middle lobe and lingular more common in men with COPD or in thin, older women; indolent symptoms
Swyer-James syndrome focal or unilateral bronchial wall thickening, increased lucency in the small lung due to air trapping
Congenital
Cystic fibrosis upper lung zone, central extensive cystic and cylindrical bronchiectasis
Primary ciliary dyskinesia middle lobe and lingular situs inversus, chronic sinusitis, and bronchiectasis
Mounier-Kuhn syndrome central absence or atrophy of the elastic fibers and smooth muscle in trachea and main bronchial wall
Williams-Campbell syndrome midorder bronch cartilage deficiency in the midorder bronchi
Bronchial atresia focal or unilateral most common location
Fibrosing Diffuse Disease
Sarcoidosis upper lung zone, central bilateral hilar lymphadenopathy, perilymphatic nodules
Pulmonary fibrosis lower lung zone, peripheral varicoid bronchiectasis, architectural distortion, honeycombing in UIP
ARDS middle lobe and lingular varicoid bronchectasis, diffuse ground-glass opacities
Endobronchial/Peribronchial Lesions
Endobronchial tumour focal or unilateral
Foreign body focal or unilateral
Stricture focal or unilateral
Other
Bronchiolitis obliterans diffuse significant cause of post-transplantation morbidity and mortality; diffuse air trapping

Management

Acute Exacerbation

  • Send sputum cultures every time
  • Cover empirically with usual pneumonia treatment x 14 days
  • Add Pseudomonas or MRSA coverage if needed
  • Consider prednisone if asthma or ABPA

Chronic Management

  • Chest physiotherapy it most important
  • Mucous (questionable utility outside of CF)
    • Hypertonic saline
    • Mannitol
    • DNAse/Pulmozyme is BAD outside of CF
    • Mucomyst
  • Antiinflammatory
    • Consider inhaled bronchodilators if responsive
    • Consider azithromycin 500mg po MWF
  • Inhaled antimicrobials, if colonozed with Pseudomonas and more than 3 exacerbations annually
    • Inhaled tobramycin
    • Inhaled colistin
    • Possibly inhaled ciprofloxacin
  • Surgery: resection or transplantation can sometimes be considered
  • Supportive
    • Annual flu vaccine
    • Smoking cessation
    • Pulmonary rehabilitation
    • GERD treatment

Further Reading

References

  1. ^  Bethany Milliron, Travis S. Henry, Srihari Veeraraghavan, Brent P. Little. Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases. RadioGraphics. 2015;35(4):1011-1030. doi:10.1148/rg.2015140214.