Week Three - Case Four

Question 1

what is bronchiectasis

Answer 1

an obstructive lung disease

It is almost always a result of bronchial obstruction leading to infection with inflammation (distal to the obstruction)

Question 2

what causes bronchiectasis

Answer 2

Cystic fibrosis – the most common cause in developed countries

Bordetella pertussis infections (Whooping Cough) can sometimes cause bronchiectasis later in life

Ciliary dysfunction syndromes

Primary hypogammaglobuminaemia

Congenital abnormalities (usually rare ones)

TB – this is the most common cause worldwide

Question 3

what are the symptoms of bronchiectasis

Answer 3

chronic cough

production of large amounts of foul smelling sputum - may have flecks of blood

finger clubbing may be present

recurrent RTIs

fever and malaise

haemopytsis

Question 4

what are the signs of bronchiectasis

Answer 4

May be unilateral or bilateral.

Coarse crackles (numerous) over areas containing large amounts of sputum
Possible collapse (no breath sounds)
Reduced or absent breath sounds at areas distal to places of obstruction

Question 5

what is the pathology behind bronchiectasis

Answer 5

there will be destruction of the alveolar walls (and the elastin contained in them), with fibrosing of the remaining parenchyma.

Question 6

what will then happen to the airway

Answer 6

the airway will then dilate as the surrounding scar tissue contracts. this can in itself cause secondary inflammatory changes which leads to further destruction of the airwaysw

Question 7

where in the lungs is most greatly affected

Answer 7

usually the lower lobes are most greatly affected. this can lead to pooling of bronchial secretions, which increases the risk of further infections in this area. there will also be collections of pus

Question 8

what may you find in a sputum sample

Answer 8

you may find pseudomonas aeruginosa, H influenzae, staph aureus, aspergillus fumigates, fungi (e.g. Aspergillus) and various mycobacteria

Question 9

what may u see on a CXR in advanced disease

Answer 9

In advanced disease you may be able to see areas of thickened airway walls, cystic spaces, and consolidation or collapse.

Question 10

what is the management of bronchiectasis the same as

Answer 10

the management of CF

Question 11

what is essential in the management of bronchiectasis

Answer 11

Postural drainage – this is essential! Patients should be trained by physiotherapists in how to tip themselves into a position in which the affected lobe(s) is drained in an uppermost direction at least 3 times a day for 10-20 minutes – most patients find the most effective position is to lie down over the side of the bed with the head and thorax down

Question 12

what are the most common antibiotics given in bronchiectasis

Answer 12

to halt progression of the disease you need to give adequate antibiotic therapy. This obviously depends on the infective organism:

Initially, try a broad spectrum, such as cefaclor or ciprofloxacin.

Try flucloxacillin 500mg every 6 hours if this fails. This is to treat staph aureus.

If the sputum remains yellow or green after regular phsyio and other AB’s, then it is likely the infective organism is P aeruginosa. This probably required inhaled AB’s, such as ceftazidime (which can also be given IV). You could also try inhaled cipro, but this creates resistance quickly.

Question 13

what else may be given in the management of bronchiectasis (3 things)

Answer 13

steroids

bronchodilators

surgery

Question 14

what are the complications of bronchiectasis

Answer 14

pneumonia
abscess
empyema
pulmonary fibrosis
cor pulmonale
metastatic absences in the brain

Question 15

what plaques may form as a result of bronchiectasis

Answer 15

Amyloid plaques are insoluble fibrous protein aggregates. Their accumulation can lead to amyloidosis. A protein as described as being amyloid if it has a slightly altered structure, making it insoluble.
Amyloidosis can occur in many organs, and the symptoms vary greatly from organ to organ

Question 16

what is the prognosis of bronchiectasis

Answer 16

This has greatly improved with antibiotic therapy. Ultimately, many patients die from respiratory failure due to deterioration of the lung tissue. Cor pulmonale is also common.

Question 17

what are the features consistent on a CXR with bronchiectasis

Answer 17

• tram-track opacity
• mucus plugging
• ring shadow
• consolidation

Question 18

what is bronchiectasis

Answer 18

is a chronic condition in which the bronchial tree becomes abnormally and irreversibly dilated. the bronchial walls become thickened and inflamed, leading to a build-up of excess mucus which can make the lungs vulnerable to infection

Question 19

what are the causes of non-CF bronchiectasis

Answer 19

Idiopathic (most common)

Post-infective – e.g., bacterial pneumonia, mycobacterial infection

Allergic – e.g., to mould or fungus, especially in allergic bronchopulmonary aspergillosis (ABPA)

Autoimmune – associations with connective tissue diseases and inflammatory bowel disease

Immunodeficiency – e.g., primary ciliary dyskinesia, primary immunodeficiency disorders, HIV/AIDs

Obstruction – secondary to severe asthma or COPD, or distal to a neoplasm or inhaled foreign body

Question 20

what is the epidemiology of bronchiectasis

Answer 20

An estimated 212,00 people in the UK are living with non-CF bronchiectasis

There is a female to male predominance

Incidence increases with age, with 60% of diagnoses made in the over-70 age group

Interestingly, bronchiectasis is more commonly diagnosed in the least deprived sections of the UK population, in contrast to other respiratory disorders

Question 21

what is the investigations for Cystic fibrosis

Answer 21

sweat test and CFTR genetic testing

Question 22

what does bronchoscopy test for

Answer 22

lung cancer

Question 23

what does total IgE test for

Answer 23

ABPA

Question 24

what does decal calprotectin test for

Answer 24

inflammatory bowel diseasew

Question 25

what does immunoglobulins (IgG, IgA and IgM test for)

Answer 25

immunodeficiency

Question 26

what is the treatment plan for bronchiectasis

Answer 26

Physiotherapy and airway clearance techniques

Cough assist devices

Smoking cessation: it is important for healthcare professionals to know what services are available to refer patients to, do you know how to find this information?

Pneumococcal and annual influenza vaccinations

Treatment with short courses of antibiotics for exacerbations or chest infections

In rare cases surgery, for example a lobectomy if bronchiectasis is localised and symptoms are debilitating

Question 27

what can treat Moraxella catarrhalis and what is it resistant to

Answer 27

it is sensitive to co-amoxiclav and resistant to amoxicillin

Question 28

what is the sail sign

Answer 28

a dense triangular opacity overlying the cardiac shadow

Question 29

what is classed as an exacerbation of bronchiectasis

Answer 29

defined as deterioration in three or more of the following key respiratory symptoms for at least 48 hours

• cough
• sputum volume/consistency
• sputum purulence
• breathlessness
• fatigue and or malaise
• haemopysis