Bronchiectasis for PACES

The patient appears cachexic.
There is finger clubbing.
On auscultation of the chest there are crackles.

Bonus points: look for evidence of cor pulmonale.

Recap of Part 1 and Part 2 notes on bronchiectasis here

Remember the 4 main PACEs differentials of clubbing and crackles are:

1. bronchiectasis
2. lung abscess
3. bronchial carcinoma
4. fibrosing alveolitis

What is bronchiectasis?

Bronchiectasis is a condition in which recurrent or persistent bronchial sepsis leads to irreversibly damanged and dilated bronchi.  Clinically this manifests as a chronic cough often productive of large quantities of purulent sputum.

How would you investigate a patient with bronchiectasis?

I would do a baseline CXR and a HRCT, looking for the characteristic 'signet ring' of a dilated bronchi larger than the accompanying vascular bundle.  I would also investigate possible causes, so sweat test and CTFR gene mutation looking for CF, serum immunoglobulins and electrophoresis looking for hypogammaglobulinaemia, IgE to aspergillus looking for anti broncho-pulmonary aspergillosis, ciliary investigation with the saccharin test and bronchoscopy to exclude proximal obstruction.  Lung function tests and sputum culture would also be helpful in guiding future management.

How would you treat bronchiectasis?

The mainstay is good physiotherapy to teach airway clearance techniques.  Bronchodilators have a role if there is airway obstruction.  Antibiotics should be given in exacerbations, and long-term antibiotics should be considered if there are 3 or more exacerbations a year.  Occasionally lung resection may be considered, for example if there is localised disease not adequately managed with medical treatment.

What causes of bronchiectasis do you know?

Post-infection, for example after pneumonia or pertussis, inflammatory, for example after aspiration, congenitial, such as CF or Kartageners, immune-mediated, for example due to hypogammaglobulinaemia or allergic broncho-pulmonary aspergillosis.