Bronchiectasis: Difference between revisions

Revision as of 10:15, 26 May 2021

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)
- Congenital
  - Bronchopulmonary sequestration
  - Williams-Campbell syndrome (cartilage deficiency)
  - Mouner-Kuhn syndrome (tracheobronchmegaly)
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

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

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

@@ Line 1: / Line 1: @@
-== Definition ==
+== Background ==
 * Permanent dilatation of the small airways, causing persistent microbial infection
-== Etiology ==
+=== Etiologies ===
 * Focal
@@ Line 9: / Line 9: @@
 *** Chronic infection
 **** Granulomatous
-***** Tuberculosis
+***** [[Tuberculosis]]
-***** Histoplasmosis
+***** [[Histoplasmosis]]
-***** MAC/NTM
+***** [[MAC]]/[[NTM]]
-**** Bacterial: S. aureus, Klebsiella, Pseudomonas
+**** Bacterial: [[Staphylococcus aureus]], [[Klebsiella]], [[Pseudomonas]]
 **** Viral
 *** Obstruction
@@ Line 18: / Line 18: @@
 **** Tumour
 *** Right middle lobe syndrome
-*** Swyer-James-MacLeod syndrome (postinfective obliterative bronchiolitis)
+*** [[Swyer-James-MacLeod syndrome]] (postinfective obliterative bronchiolitis)
 ** Congenital
 *** Bronchopulmonary sequestration
-*** Williams-Campbell syndrome (cartilage deficiency)
+*** [[Williams-Campbell syndrome]] (cartilage deficiency)
-*** Mouner-Kuhn syndrome (tracheobronchmegaly)
+*** [[Mouner-Kuhn syndrome]] (tracheobronchmegaly)
 * Diffuse
 ** 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
-*** 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
 ** Near drowning
 ** Toxic inhalation
 ** Miscellaneous
-*** Yellow nail syndrome
+*** [[Yellow nail syndrome]]
 *** Postradiation traction bronchiectasis
-*** IPF
+*** [[IPF]]
-== Epidemiology ==
-== Risk Factors ==
 == Clinical Manifestations ==
-=== History ===
 * Chronic cough (98%)
@@ Line 59: / Line 53: @@
 * Hemoptysis (27%)
 * Recurrent pleurisy (20%)
-=== Signs &amp; Symptoms ===
 == Investigations ==
 * 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
 ** Sweat chloride testing +/- genetic testing to rule out CF
@@ Line 81: / Line 73: @@
 == Management ==
-=== Acute exacerbation ===
+=== Acute Exacerbation ===
 * Send sputum cultures every time
@@ Line 88: / Line 80: @@
 * Consider prednisone if asthma or ABPA
-=== Chronic ===
+=== Chronic Management ===
 * Chest physiotherapy it most important
@@ Line 109: / Line 101: @@
 ** Pulmonary rehabilitation
 ** 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]]