Bronchiectasis

Defined as inflamed, easily collpsible airways, with obstruction on PFTs. Dx by chronic sputum, bronchial wall thickening, and luminal distension on CT.

Clinical features are cough, sputum for months to years. Dyspnea, wheeze, pleuritic c/p, hemoptysis also seen.

Pathophysiology is acquired disorder of bronchi and bronchioles; abnormal dilation of walls and scarring.

Causes:

Focal: Foreign body, TB, old infection (usually a serious pneumonia)

Global: cystic fibrosis, dysmotile cilia (Kartagener's), allergic bronchopulmonary aspergillosis, nontuberculous mycobacterial infection, alpha-1 anitrypsin deficiency, RA, IBD

NB- "Traction bronchiectasis" is common in pulmonary fibrosis.


Investigations:

1) CXR, high-resolution CT
2) PFTs when stable
3) ECG (for cor pulmonale)
4) CBC, immunoglobulins, Ig subclasses (esp for IgE- ABPA), RF, alpha-1 antitrypsin, HIV
5) Sputum for bacteria, AFB (NB- nontuberculous mycobacteria), fungi
6) Sweat Cl +/- genetic testing
7) TB skin test
8) Aspergillus precipitins (for allergic bronchopulmonary aspergillosis)
9) Ciliary studies (e.g. EM)

CXR- tram lines

CT- dilated airways (more than 1.5x accompanying vessel), thickened bronchial walls, cysts, 'signet ring sign'


Exacerbations

Heralded by increased sputum, SOB, pleuritic pain, hemoptysis. Rarely do you see a new infiltrate.

Organisms: H. Flu, pseudomonas, st. pneumo. Fluoroquinolone is reasonable 1st line.

Prevention: several variations on chronic Abx, possibly inhaled tobramycin.

Chest physio is vital; hydration, mucolytics, bronchodilators all have role.


Reference:

Click here for a review from Chest on bronchiectasis