BRONCHIECTASIS

Question 1

What is bronchiectasis?

Answer 1

Persistent or progressive chronic debilitating disease characterized by permanent dilation of the bronchi due to irreversible damage to the elastic and muscular components of the bronchial wall.
It can be focal (limited to one area of the lung) or diffuse

Question 2

How does bronchiectasis present?

Answer 2

Symptoms can vary from intermittent expectoration and infection, to persistent daily expectoration of large volumes of purulent sputum.
Dyspnoea
Fever
Fatigue, reduced exercise tolerance
Haemoptysis
Rhinosinusitis
Weight loss
Chest pain

Question 3

What are signs of bronchiectasis?

Answer 3

abnormal chest auscultation- coarse crackles and wheeze, high-pitched inspiratory squeaks
Palpable chest secretions on coughing or forced expiratory manoeuvre
clubbing may be present

Question 4

What can cause bronchiectasis?

Answer 4

Idiopathic up to 40% of cases
1 in 3 cases are associated with a severe lung infection in childhood e.g. pneumonia, whooping cough, TB, measles
Immunodeficiency - IgA deficiency, IgG deficiency, , hypogammaglobulinaemia, HIV, malignancy
Allergic bronchopulmonary aspergillosis
Aspiration
CF
Cilia abnormalities e.g. primary ciliary dyskinesia, youngs syndrome
Connective tissue diseases - RA, Sjögren’s syndrome, Crohn’s, UC

Most commonly caused by a previous severe LRTI e.g. pneumonia or TB
post-infective: tuberculosis, measles, pertussis, pneumonia
bronchial obstruction e.g. lung cancer/foreign body/mucus plugging/lymph nodes
Mucociliary clearance dysfunction - primary ciliary dyskinesia, young syndrome, CF
Congenital airway defects - Williams-Campbell syndrome, mounier-Kuhn syndrome, youngs syndrome, Marfan syndrome
Others: allergic bronchopulmonary aspergillosis (ABPA), rheumatic diseases, COPD, IBD, yellow nail syndrome

Question 5

Whats the epidemiology of bronchiectasis?

Answer 5

More common in females than males
60% of cases diagnosed over the age of 70 (>1% of over 70s have it)
Up to 25% of those with COPD might actually have bronchiectasis

Question 6

Whats the pathophysiology of bronchiectasis?

Answer 6

Infective insults -> bronchial dilatation and mucociliary impairment -> infections and inflammation -> ongoing inflammatory cycle and further infections -> permenant airway remodelling which is dilatation of the airways with excessive mucous production

Question 7

What are the most frequent pathogens found in bronchiectasis?

Answer 7

Haemophilus influenzae (20–40%)
Pseudomonas aeruginosa (10–30%),
Moraxella catarrhalis
Streptococcus pneumoniae, Staphylococcus aureus
Enterobacteriaceae
Non-tuberculous mycobacteria (NTM) accounts for 1–10% of cases.

Question 8

Whats the prognosis of bronchiectasis?

Answer 8

People with mild bronchiectasis can have a normal life expectancy
Worse prognosis if extensive disease, have frequent severe exacerbations, dyspnoea, primary antibody deficiency disorder, colonisation with pseudomonas, comorbidity, smokers

Mortality rates are 2 fold higher than general population

Question 9

Whats the strongest predictor of mortality in bronchiectasis?

Answer 9

Dyspnoea
Pseudomonas colonisation

Question 10

What are complications of bronchiectasis?

Answer 10

Infective exacerbations and chronic bacterial colonisation
Haemoptysis
Pneumothorax
Respirator failure
Cor pulmonary
Chest pain
IHD
Anxiety and depression
Urinary incontinence
Nutritional deficiency
Fatigue and reduced exercise tolerance
Reduced QOL

Question 11

When should you suspect an infective exacerbation of bronchiectasis?

Answer 11

There is a change in one or more of the common symptoms: increased sputum volume or purulence, worsening dyspnoea, increased cough, increased fatigue/malaise.
New symptoms develop, such as fever, pleuritic pain, or haemoptysis

Question 12

How is bronchiectasis diagnosed?

Answer 12

CXR - may be normal or show non-specific abnormalities e.g. tram-track airways and ring shadows
Thin section CT - shows dilated bronchi with signet ring sign

Question 13

What is the signet ring sign?

Answer 13

seen in bronchiectasis when the dilated bronchus and accompanying pulmonary artery branch are seen in cross-section. The bronchus and artery should be the same size, whereas in bronchiectasis, the bronchus is markedly dilated.

Question 14

What investigations should be done for bronchiectasis?

Answer 14

Sputum culture
CXR
Spirometry
Oxygen sats
FBC
Others dependant on probable underlyign cause e.g. testing for primary ciliary dyskinesia, CF testing, serum total immunoglobulin etc
High resolution CT (thin section scanning)

Question 15

How do you exclude allergic bronchopulmonary aspergillosis as a DDx for bronchiectasis?

Answer 15

Test serum total immunoglobulin - IgE and skin prick test to aspergillosis

Question 16

Which pt with bronchiectasis should be followed up in secondary care?

Answer 16

Anyone at high risk of future exacerbations e.g.
had 3 or more exacerbations in a year
Chronic Pseudomonas aeruginosa, MRSA, or non-tuberculous mycobacteria colonization
Deteriorating bronchiectasis with declining lung function or advanced disease
Allergic pulmonary aspergillosis
Long term antibiotic therapy
Associated RA, immune defieicny, IBD, allergic bronchopulmonary aspergillosis, primary ciliary dyskinesia
Advanced disease and those considering lung transplantation

Question 17

What tool can be used to determine the mortality and exacerbation rates in bronchiectasis?

Answer 17

Bronchiectasis severity index
(Takes into account age, BMI, FEV1%, previous hopsital admission, number of exacerbations in pervious year, MRC breathlessness score, pseudomonas colonisation, colonisation with others organisms, radiological severity)

Question 18

What should be included in the annual review for bronchiectasis?

Answer 18

An assessment of symptoms and how they affect their daily lives — offer pulmonary rehabilitation to people with breathlessness that affects their daily lives.
Measurement of BMI — if the person’s BMI is less than 20 seek specialist nutritional advice.
The exacerbation frequency — refer people with three or more infective exacerbations a year, and people with fewer exacerbations that are causing significant morbidity to a respiratory specialist. May be considered for long-term prophylactic antibiotic treatment.
Taking a sputum sample for bacterial culture — specialist follow up is required for people with chronic colonization with Pseudomonas aeruginosa, opportunist Mycobacteria, or MRSA.
The Medical Research Council dyspnoea scale.
Spirometry.
Oxygen saturation levels.
Compliance with sputum clearance exercises.

Question 19

How should you manage a person with an infective exacerbation of bronchiectasis?

Answer 19

Arrange hopsital admission if deemed needing IV antibiotics
Consider other diagnoses
Consider previous antibiotic use which may ahve led to resistant bacteria
Send sputum for culture and sensitivity testing before starting antibiotics but don’t delay treatment waiting for results
7-14 days of antibiotics
Ensure there’s a suitable airway clearance technique taught by a respiratory physiotherapist
Review response to empirical treatment when sputum culture and sensitivity results are available - if not clinical improvement, modify antibiotics
If significant breathlessness you can offer a trial of LABA

Consider prescribing a course of antibiotics for people to keep at home to facilitate prompt treatment of future exacerbations

Question 20

What antibiotic treatment is suitable for an acute exacerbation?

Answer 20

Previous microbiology cultures should guide choice

Amoxicillin is first line if not available

Question 21

Consider prescribing a course of antibiotics for people to keep at home to facilitate prompt treatment of future exacerbations

Answer 21

Do not routinely offer antibiotic prophylaxis to prevent acute exacerbations of bronchiectasis
If pt has 3 or more exacerbations per year they may be offered antibiotic prophylaxis e.g azithromycin (inhaled colistin if concurrent pseudomonas aeruginosa infection)

Question 22

How does cystic fibrosis cause bronchiectasis?

Answer 22

Mutation in CFTR gene -> codes for Cl- channels which help facilitate Cl- out into the airway lumen -> water is reabsorbed together with Na+ -> thick mucous -> impaired mucociliary apparatus so impaired mucus clearance -> increases risk of infections and persistent inflammation

Question 23

How does airway obstruction cause bronchiectasis? Explain in detail for a doctor

Answer 23

Airway obstruction -> endothelial damage -> mucociliary apparatus dysfunction -> increased risk of infection and inflammation -> chronic inflammation -> remodelling of bronchi

Question 24

What is primary ciliary dyskinesia?

Answer 24

a rare, autosomal recessive genetic ciliopathy, that causes defects in the action of cilia lining the upper and lower respiratory tract, sinuses, Eustachian tube, middle ear, Fallopian tube, and flagella of sperm cells.

Within the first few months of life, most develop a chronic mucus-producing cough and runny nose.[3] The main consequence of impaired ciliary function is reduced or absent mucus clearance from the lungs, and susceptibility to chronic recurrent respiratory infections

Question 25

What is Kartagener’s syndrome?

Answer 25

A triad:
Situs inversus (transposition of the viscera)
Abnormal frontal sinuses (producing sinusitis and bronchiectasis)
Primary ciliary dyskinesia

Question 26

Why can hypogammaglobulinaemia cause bronchiectasis?

Answer 26

Defieicny of immunoglobulins -> increased susceptibility to infections of respiratory tract -> chronic inflammation and tissue damage

Question 27

What is allergic bronchopulmonary aspergillosis? How can it cause bronchiectasis?

Answer 27

A hypersensitivity reaction to Aspergillus which typically affects pt with asthma or CF
It causes inflammation and damage to the airways which can leads to bronchiectasis

Question 28

What will be found upon sputum sampling in bronchiectasis?

Answer 28

Neutrophilia (although in 25% of cases it may be eosinophilia)
Presence of bacteria
Increased mucus production

Question 29

What is chronic colonisation?

Answer 29

the persistent presence of bacteria in the airways of individuals with bronchiectasis, even in the absence of acute infections. In bronchiectasis, the airways are often damaged and widened, which can result in the accumulation of mucus and secretions that can harbor bacteria. Chronic colonization can contribute to ongoing airway inflammation and exacerbations, and may also increase the risk of developing antibiotic resistance.

Question 30

What are the most common bacteria associated with chronic colonisation in bronchiectasis?

Answer 30

Pseudomonas aeruginosa, Haemophilus influenzae, and Streptococcus pneumonia, Aspergillus, non-TB mycobacterium

Question 31

What are the 3 most common causes of bronchiectasis in the UK?

Answer 31

Having had a lung infection in the past that damaged bronchi
Underlying problems with the immune system
Aspergillosis

Question 32

What is tram track lines on CXR or CT?

Answer 32

Thickened bronchial walls

Question 33

Whats gold standard for diagnosing bronchiectasis?

Answer 33

CT high resolution

Question 34

Why are macrolide antibiotics best for bronchiectasis?

Answer 34

They have anti-inflammatory effects, immunomodulatory effects, broad-spectrum activity and low risk of resistance

Question 35

When may surgical resection of a lung be indicated?

Answer 35

Localised disease that has failed to respond to medical therapy
Massive haemoptytsis
Lung cancer
Aspergillosis

Question 36

How do you manage Allergic bronchopulmonary aspergillosis?

Answer 36

Oral glucocorticoids

Question 37

What is allergic bronchopulmonary aspergillosis?

Answer 37

a fungal infection of the lung due to a hypersensitivity reaction to antigens of Aspergillus fumigatus after colonization into the airways
It predominantly affects pt with bronchial asthma and CF

Question 38

What are the features of Allergic bronchopulmonary aspergillosis?

Answer 38

Bronchoconstriction - previous asthma or new wheeze/cough/dyspnoea
Recurrent chest infections
Bronchiectasis

Question 39

What investigations should you do for Allergic bronchopulmonary aspergillosis?

Answer 39

eosinophilia
CXR
positive radioallergosorbent test to Aspergillus
positive IgG precipitins
raised IgE

Question 40

How do you manage allergic bronchopulmonary aspergillosis?

Answer 40

Oral glucocorticoids