Bronchiectasis and Cystic Fibrosis

Bronchiectasis: abnormal, permanent thickening and dilatation of the bronchi and bronchioles due to repeated infection, impaired drainage, airway obstruction +/- defective host response.



  • Shortness of breath
  • Chronic productive cough: thick, smelly, purulent sputum
  • Haemoptysis
  • Recurrent infections
  • Pleuritic chest pain
  • Weight loss
  • History of childhood infections, sinusitis, subfertility



On examination:


On inspection:

  • Purulent sputum in pot next to bedside
  • Inhalers by bedside


On examination:

  • Clubbing
  • May have scars
  • Reduced chest expansion
  • Early coarse inspiratory crepitations that alter with coughing but do not quieten/disappear on leaning forwards
  • Wheeze, inspiratory clicks


Comment on whether there is evidence of complications such as respiratory failure (oxygen, cyanosis, CO2 retention flap) or cor pulmonale (RV heave, loud P2, raised JVP, peripheral oedema) or infection (bronchial breathing, antibiotics at bedside, fever)


Tell the examiner that you suspect bronchiectasis affecting the upper/lower zones


Comment on whether there are signs of a possible underlying cause e.g:

  • Cystic Fibrosis: young, thin, short, PEG, portacath/Hickmann, signs of chronic liver disease
  • Kartagener’s Syndrome: Dextrocardia
  • Connective tissue disease: peripheral symmetrical deforming polyarthropathy, cutaneous signs of lupus
  • Yellow Nail Syndrome: yellow nails, lymphoedema

Comment on whether there are signs of treatment:


  • Steroid usage: Cushingoid, thin skin, bruising, proximal myopathy
  • Oxygen
  • Scars- eg. from lung surgery
  • Inhalers, nebulisers, antibiotics


Comment that your differential diagnosis for clubbing and crepitations includes:


  1. Interstitial lung disease: Fine late end-inspiratory crepitations that do not clear with coughing but do quieten/disappear on leaning forwards
  2. Lung Cancer: cachexia, tar staining, lymphadenopathy
  3. Abscess


Other causes of crepitations include:


  • Pneumonia
  • Heart Failure


Severity is based on:

  • MRC dyspnoea scale >4
  • Number of bronchopulmonary segments involved
  • Lung function assessment


Causes of bronchiectasis include:

  • Post infective: bacterial (e.g. pertussis, TB) and viral (e.g. measles, HIV) and ABPA, recurrent aspiration
  • Congenital mucociliary clearance defects: Cystic fibrosis, Kartageners syndrome (dextrocardia/complete situs inversus, sinusitis, infertility)
  • COPD
  • Immunodeficiency – HIV, hypogammaglobulinema
  • Mechanical – bronchial obstruction with foreign body, carcinoma, lymph node, granuloma
  • Connective tissue disease- RA, SLE
  • Other: yellow nail syndrome (lymphoedema and pleural effusion), Youngs syndrome (mercury intoxication, infertility), IBD, congenital kyphoscoliosis, idiopathic, fibrosis-traction bronchiectasis




Bedside tests:


ABG (respiratory failure)

ECG (right heart strain)

Laboratory tests:


Bloods: FBC (white cell count, eosinophilia, anaemia), CRP, Immunoglobulins (decreased in hypogammaglobulinema), autoimmune screen (ANA, RF, anti-CCP), HIV test, Aspergillus precipitins and IgE (ABPA), quantiferon


Sputum MC+S, AFB and cytology




CXR to diagnose, assess extent and distribution, complications and cause

  • Tramlines (diseased bronchi side on)
  • Ring shadows (diseased bronchi end on)
  • Gloved finger: mucoid impactions in large air ways
  • Hyperinflation
  • 7% normal CXR


HRCT to diagnose, assess extent and distribution and severity, complications and cause and exclude differentials.  Much more sensitive than CXR.  This is the diagnostic test of choice.

  • Signet ring sign- thickened end-on dilated bronchus >1.5 times larger than adjacent pulmonary artery
  • Tram tracking
  • Ring shadows
  • Volume loss
  • Flame and blob sign: mucus plugging


Special tests:


Lung function tests: Obstructive pattern

Video assisted, open or transbronchial biopsy to look at histology to exclude malignancy

Echo: to assess for pulmonary hypertension

CF: sweat test, genotyping

Cilia studies





Educate patient, stop smoking, vaccines (influenza, pneumococcal), chest physiotherapy and postural drainage and inspiratory muscle training, optimise nutrition



Treat the cause e.g. immunoglobulins in hypogammaglobulinemia

Antibiotics: acute and prophylactic (oral/iv/nebulised)




Manage complications e.g. infection, haemoptysis, cor pulmonale



Lobectomy/bullectomy- localised disease

Lung transplant e.g. in cystic fibrosis




Complications of bronchiectasis:




Recurrent infection

Haemoptysis – massive >400mls


Cor pulmonale





Cerebral abscess

Secondary amyloidosis



Major pathogens associated with bronchiectasis:


Pseudomonas aeruginosa

Haemophilus influenzae



And also:

Staph aureus



Burkholderia cepacia in CF patients



Cystic Fibrosis:


  • Multisystem disease
  • Autosomal recessive disease due to defect in CFTR gene located on chromosome 7
  • Most common mutation is delta F508
  • CFTR protein is present on all exocrine glands (it codes for an epithelial chloride channel)
  • Defective CFTR protein leads to thick viscous secretions which enable colonization and infection and block lumens
  • Mean survival age 32 years


Systems affected/associations of cystic fibrosis include:


  • GI: Distal intestinal Obstruction Syndrome
  • Pancreatic enzyme insufficiency with fat and protein malabsorption
  • Diabetes
  • Gallstones
  • Biliary cirrhosis leading to portal hypertension
  • Subfertility (men)
  • Sinus disease/nasal polyps
  • Osteoporosis


Organisms most likely to colonise CF patients:


  • Staphylococcus aureus
  • Haemophilus influenzae
  • Pseudomonas species
  • Burkholderia cepacia – worse prognosis and possible contraindication to transplantation


Tips – clues to diagnose cystic fibrosis:


  • Young, short stature, thin
  • Clubbing often present
  • PEG feeding, long term vascular access device
  • Ask about other systems- GI and endocrine




Written by Dr Amna Shah

Edited by Dr Sarah Kennedy


Resources used to write this document are listed in the references section of this webpage