13 - Bronchiectasis and Cystic Fibrosis Flashcards
What is bronchiectasis?
Chronic irreversible dilatation of one or more bronchi. Bronchi has poor mucus clearance and there is a predisposition to recurrent or chronic bacterial infection
(Aetiology of chronic inflammation damaging elastic and muscular walls)
What is the gold standard diagnostic test for bronchiectasis and what would the result of this test be?
- High resolution CT
- Bronchi bigger than adjacent vessels (pulmonary artery so signet ring sign)
- Bronchial wall thickening (tram tracks)
- Take a good history, e.g TB or infections as a kid?
What are some of the clinical symptoms of bronchiectasis?
- Chronic cough and daily sputum production
- Breathlessness on exertion
- Intermittent haemophysis
- Nasal symptoms
- Chest pain
- Fatigue
Key is history as symptoms are vague
What are some clinicial signs of bronchiectasis?
- Hypoxaemia
- Fever
- Haemoptysis
- Fine crackles (rales - popping alveoli)
- Rhonchi (low pitched like snoring)
- High pitch inspiratory wheeze
- Crackles and wheezes
- Weight loss
- Clubbing fingers
What are some causes of bronchiectasis?
- Post infective: whooping cough and TB
- Immune deficiency: hypergammaglobulin
- Genetic/Mucociliary clearance defects: CF, primary ciliary dyskinesia, Young’s syndrome, Kartagener syndrome
- Obstruction: foreign body, tumour, extrinsic lymph node
- Idiopathic
What is Young and Kartagener syndrome?
Youngs: triad of sinusistis, bronchiectasis and reduced fertility (obstructive azospermia)
Kartagener: triad of sinusitis, bronchiectasis and situs invertus
What are the common organisms in bronchiectasis?
- H.Influenzae
- Pseudomonas aeruginosa
- Moraxella atarrhalis
- Strenotrophomonas maltophilia
- Fungi: aspergillus and candida
- Non tuberculosis mycobacteria
- Less common = Staph Aureus
What causes a viscious cycle of bronchiectasis and why do we need to catch it early?
- Bronchial dilation leads to mucus accumulation so impaired mucus clearance and increased risk of infection
- Infection leads to inflammation and loss of elastic fibres and bronchial smooth muscle so more dilatation
- Need to catch early to stop spread over whole lung as irreversible
Is bronchiectasis restrictive or obstructive?
- Obstructive so will see when doing lung function tests so can monitor progression
How can you tell the difference between chronic bronchitis and bronchiectasis?
How is bronchiectasis managed?
- Treat underlying cause
- Physiotherapy to clear mucus
- Antibiotics based on sputum cultures for exacerbations
- Chronic suppressive therapy
- Supportive: flu vaccine, bronchodilators
- Pulmonary rehab if MRC dysponea >3
What is cystic fibrosis?
Autosomal recessive disorder leading to mutations in the CFTR. Can lead to multisystem disese (usually resp and GI tract) characterised by thick secretions
Main mutation is Phe508del
How do we diagnose cystic fibrosis?
- Phenotypic
- Sweat test
- Genetic testing
What are the common CF presentations?
- Meconium Ileus: bowel obstructive so bilious vomiting, abdominal extension and delay in passing meconium
- Intestinal malabsorption: lack of pancreatic enzymes
- Recurrent chest infections
- Doesn’t normally present as screened for in newborns
Why may a patient not be diagnosed with CF until adulthood and what conditions may make you suspect CF in an adult?
May have mutation 5 where they just have a reduced synthesis of CFTR
Suspect in idiopathic pancreatitis, recurrent sinusitis, lung infections, infertility, allergic bronchopulmonary aspergilliosis
What are some common complications with CF?
- Infertility
- Gall stones
- Cardiac failure
- Chronic liver disease
- Osteoporosis
- Arthritis
- Respiratory failure (P.aeruginosa)
What is some lifestyle advice a GP may give to a CF patient?
How is CF managed?
- MDT
- Holistic care
- Pneumococcal and Influenza vaccinations
- Optimal nutritional state
- Airway clearance
What is a newly approved drug that is used for patients with CF?
Orkambi - lumacaftor and ivacaftor
