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