Respiratory V (Bronchiectasis; Respiratory Failure) Flashcards
Define what is meant by bronchiectasis [1]
Bronchiectasis is the abnormal dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall
Which inherited diseases can cause bronchiectasis? [4]
- (Alpha-1-antitrypsin deficiency)
- Connective tissue disorders (e.g., rheumatoid arthritis)
- Cystic fibrosis
- Yellow nail syndrome
Describe the classic triad of yellow nail syndrome [3]
- Yellow fingernails
- Bronchiectasis
- Lymphoedema
TOM TIP: Yellow nail syndrome is characterised by yellow fingernails, bronchiectasis and lymphoedema. Patients are stable and have good clinical signs, making it a good choice for OSCEs. As it is rare, examiners will score high marks if you can combine these features and name the diagnosis.
Describe the pathophysiology of bronchiectasis [7]
Cole’s ‘vicious cycle hypothesis:
A deficit in mucociliary clearance +/- immune function
→
Microorganism acquisition, colonisation and infection
→
Chronic inflammation
→
Dilation and thickening of bronchi
→
Bronchial wall oedema and increased mucus production
→
More infections
→
Further inflammation and damage.
Describe the three morphological types of bronchiectasis [3]
Tubular/cylindrical:
- most common type
- the morphology is of smooth uniform dilatation of the bronchi with loss of normal tapering. **
- This type is associated with the ‘signet ring sign**’ due to an increased bronchoarterial ratio and the ‘tram-track sign’ due to parallel bronchial walls.
Varicose:
- relatively uncommon, the bronchi are irregular with dilatation interspersed with areas of constriction.
Cystic:
- associated with cystic fibrosis, dilated bronchi that are cyst like and filled with either air or fluid.
Describe the classical symptoms of bronchiectasis [5]
- Shortness of breath
- Chronic productive cough: may produce large amounts of purulent sputum and sometimes haemoptysis
- Recurrent chest infections
- Weight loss
- Fever
- Chest pain
- GORD
Describe the classical signs of bronchiectasis [5]
- Scattered crackles throughout the chest that change or clear with coughing
- Scattered wheezes and squeaks
- Sputum pot by the bedside
- Oxygen therapy (if needed)
- Weight loss (cachexia)
- Finger clubbing
- Signs of cor pulmonale (e.g., raised JVP and peripheral oedema)
When taking a history and examining the patient, it is also important to consider other systems of the body too, as these may reveal co-morbid conditions associated with the development of bronchiectasis
.
Which do these include? [4]
Joints:
- RA
GI:
- IBD
- Cystic fibrosis
- GORD
Describe what CT [7] and CXR [2] would reveal when investigating for bronchiectasis? [2]
High resolution chest CT
- Test of choice
- Thickened & dilated airways
- May show fluid-filled cysts; these represent superimposed infection and warrant a course of systemic antibiotics.
- Tram line or signet ring appearance
- Bronchoarterial ratio > 1: the internal airway lumen is larger than the adjacent pulmonary artery (signet ring sign)
- Lack of tapering: bronchi should taper in diameter as they travel distally from the lung hila to the periphery
- Bronchus visible within 1cm of pleural surface: normal, non-dilated airways cannot usually be seen within 2cm of the pleura
CXR:
- the majority of X-rays will be abnormal in those with bronchiectasis but findings are non-specific and further imaging is required.
- They are also useful for ruling out other possible causes such as TB or malignancy
Sputum culture is used to identify colonising and infective organisms. The most common infective organisms are? [2]
Haemophilus influenza
Pseudomonas aeruginosa
Asides from imaging investigations, describe what else you would investigate for bronchiestasis [7]
Sputum culture
- Most commonly Haemophilus influenzae and Pseudomonas aeruginosa
FBC:
- may reveal high eosinophil count in bronchopulmonary aspergillosis
specific IgE or skin prick test to Aspergillus fumigatus
serum alpha-1 antitrypsin phenotype and level
serum immunoglobulins
- to identify individual immunoglobulin deficiencies as underlying aetiology
Rheumatoid factor
Serum HIV antibody
Describe most common pattern seen on post-bronchodilator spirometry in bronchiectasis
Post-bronchodilator spirometry: most commonly an obstructive pattern is seen, although mixed, restrictive, and normal results are also possible.
How would you distinguish between bronchiectasis and:
- COPD [2]
- Asthma [2]
COPD Differences:
- Sputum more likely to be clear (except during infection)
- Smoking stronger RF
Asthma:
- Dysopnea more associated with triggers
NB: Both diseases can co-exist with bronchiectasis
TOM TIP: The key features to remember with bronchiectasis are [4]
TOM TIP: The key features to remember with bronchiectasis are finger clubbing, diagnosis by HRCT, Pseudomonas colonisation and extended courses of 7-14 days of antibiotics for exacerbations.
Describe the treament algorithm for bronchiestasis for the initial presentation? [5]
initial presentation
1ST LINE: exercise and improved nutrition.
- Including vitamin D supplementation
- Higher BMI has beneficial outcomes
- Excercise is considered form of airway clearance
PLUS –
airway clearance therapy (ACT):
- maintenance of oral hydration; percussion, breathing, or coughing strategies
- positioning and postural drainage; positive expiratory pressure devices; and oscillatory devices
- recommended for 15 to 30 minutes, 2 or 3 times daily
PLUS –
self-management plan
CONSIDER –
inhaled bronchodilator:
- salbutamol inhaled
CONSIDER –
mucoactive agent
- hypertonic saline
BMJ BP
acute exacerbation: mild to moderate underlying disease if is first or new presentation of Pseudomonas aeruginsoa
1ST LINE –
short-term oral antibiotic:
- For adults, prescribe amoxicillin 500 mg three times a day for 7–14 days
PLUS –
increased airway clearance
PLUS –
continued maintenance therapy:
- Healthy diet & exercise
- Higher BMI
- Nebulised bronchodilators
- Nebulised hyperosmolar agents, such as hypertonic saline,
Describe how treatment for bronchiectasis would be escalated in a stepwise manner if they were suffering ≥ 3 exacerbations in one year despite following the initial management?
3 or more exacerbations per year despite maintenance therapy
1ST LINE –
reassess physiotherapy ± mucoactive treatment
PLUS –
continued maintenance therapy
- Azithromycin 500 mg three times a week, or
- Azithromycin 250 mg daily, or
- Offer a minimum of 6 months treatment, but up to 1 year may be required.
CONSIDER –
long-term antibiotic
CONSIDER –
surgery:
- Surgical resection is considered in patients with localised disease whose symptoms are not controlled by optimal medical treatment
- Complete resection of the bronchiectatic area is associated with the best results
CONSIDER –
treatment of respiratory failure
BTS sugget what criteria for a lung transplantation for bronchiestasis? [3]
BTS specify the following criteria for lung transplantation:
* Aged 65 years or less and
* FEV is < 30% predicted with significant clinical instability
or
* Rapid progressive deterioration despite optimal medical management
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Streptococcus pneumoniae. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
Amoxicillin 500 mg three times daily
When giving long term antibiotic therapy to those with bronchiestasis, if people have concurrent Pseudomonas aeruginosa infection, first-line therapy is []
inhaled colistin.
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Haemophilus influenzaebeta lactam negative. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Haemophilus influenzae. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Haemophilus influenzae (beta-lactamase positive). What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
Co-amoxiclav 625 mg three times daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Pseudomonas aeruginosa. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Pseudomonas aeruginosa. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Klebsiella. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
A patient is diagnosed with bronchiestasis. Subsequent sputum sampling diagnoses them the infective agent as Klebsiella. What is the approriate first line treatment
- Co-amoxiclav 625 mg three times daily
- Amoxicillin 500 mg three times daily
- Flucloxacillin 500 mg four times daily
- Doxycycline 100 mg twice daily PLUS rifampicin (for adults)
- Ciprofloxacin 500 or 750 mg twice daily
Which vaccines are recommonded for bronchiestasis? [2]
Vaccines (e.g., pneumococcal and influenza)
[] is the usual choice for infective exacerbations caused by Pseudomonas aeruginosa
Ciprofloxacin is the usual choice for exacerbations caused by Pseudomonas aeruginosa
How long are the extended course of Abx for infective exacerbations? [1]
Extended courses of antibiotics, usually 7–14 days
Cardiac tamponade
This CXR causes hypoxia due to which underlying mechanism
V/Q mismatch
Shunt
Diffusion limitation
Hypoventilation
Increased dead space
This CXR causes hypoxia due to which underlying mechanism
V/Q mismatch
This CXR causes hypoxia due to which underlying mechanism
V/Q mismatch
Shunt
Diffusion limitation
Hypoventilation
Increased dead space
Pneumothorax causing V/Q mismatch