Bronchiectasis

Question 1

What is bronchiectasis

Answer 1

permanent dilation of the airways secondary to chronic infection or inflammation due to destruction of elastic and muscular components of bronchial wall

Question 2

How would you classify bronchiectasis

Answer 2

CF bronchiectasis
Non- CF bronchiectasis

Question 3

What are the causes of bronchiectasis

Answer 3

. Post- infective/ recurrent pulmonary infections like measles, pertussis, pneumonia
. CF
. TB
. COPD
. Aspiration
. Chronic inflammatory disease e.g. RA, Coealiac disease
. Allergic bronchopulmonary aspergillosis
. Primary ciliary dyskinesia (Kartagener’s syndrome)
. Bronchial obstruction caused by lung cancer and foreign body
. Immune deficiency

Question 4

What is CF?

Answer 4

Autosomal recessive disorder causing increased viscosity of secretions due to defect in CF transmembrane regulator

Question 5

What are the clinical features of cystic fibrosis?

Answer 5

Recurrent chest infections
failure to thrive (delayed puberty)
malabsorption (steatorrhoea)
short stature
male infertility

Question 6

How is cf diagnosed?

Answer 6

via sweat test (will have abnormally high sweat chloride)

Question 7

how is cf screened?

Answer 7

at birth via heel prick

Question 8

what is the management of cf?

Answer 8

chest physiotherapy

Question 9

what is allergic bronchopulmonary aspergillosis?

Answer 9

bronchiectasis + bronchoconstriction caused by hypersensitivity response to aspergillus fungus

Question 10

what is the clinical features of allergic bronchopulmonary aspergillosis

Answer 10

wheeze
cough
dyspnoea

Question 11

what would you find on bloods for allergic bronchopulmonary aspergillosis

Answer 11

eosinophilla
raised igE

Question 12

management of ABPA

Answer 12

oral prednisolone

Question 13

primary ciliary dyskinesia (kartagener’s syndrome) characterised by?

Answer 13

bronchiectasis + dextrocardia (or complete sinus inversus) + recurrent sinusitis

Can cause male infertility

Question 14

what deficiency of immune cause bronchiectasis

Answer 14

selective IgA (lack of IgA)
Hypogammaglobulinaemia

Question 15

How does obstructive disease cause bronchiectasis

Answer 15

mucus plugs form in airways and obstruct airways

Question 16

Most common organisms that caused bronchiectasis

Answer 16

Haemophilus influenzae- most common
Pseudomonas aeruginosa
Klebsiella spp.
Streptococcus pneumoniae

Question 17

What are the clinical features of bronchiectasis?

Answer 17

. chronic cough associated with large (copious) amounts of purulent sputum (green/ rusty colour). May be worsen by lying flat or on one side
. haemopytsis
. recurrent chest infections
. inspiratory coarse crackles on auscultation
. clubbing
. SOB (especially on exertion)
. fever
. acute exacerbation often presents with fatigue, weight loss and wheezing

Question 18

What initial investigation will you request?

Answer 18

CXR

Question 19

What would CXR show?

Answer 19

. Obscured hemidiaphragm
. Thin-walled ring shadows with or without fluid levels
. tubular or ovoid opacities
. tram lines (parallel line shadows)

Question 20

What is the gold standard confirmatory test?

Answer 20

high res chest CT

Question 21

What would the CT show?

Answer 21

shows thickened dilated airways
varicose constrictions along airways
cysts and/or tree-in bud pattern
signet ring sign
tram-track sign

Question 22

What other investigation are there?

Answer 22

. Spirometry- look for obstructive pattern (<0.7)
. Sputum MCS- to look for underlying cause- may find haemophilus influenzae (most common) or pseudomonas aeruginosa (most common in CF patients)
. Cystic fibrosis sweat test

Question 23

What would you see in CF in cystic fibrosis sweat test?

Answer 23

abnormally high chloride

Question 24

what is included in the management plan?

Answer 24

. exercise and improved nutrients
. airway clearance therapy (chest physiotherapy)
. inhaled bronchodilator (salbutamol)
. mucoactive agent e.g. neubulised hypertonic saline
. Abx- amoxicillin, vancomycin
. Immunisations

Question 25

What is included in chest physiotherapy?

Answer 25

postural drainage percussion vibration maintenance of oral hydration

Question 26

When would we do surgery

Answer 26

lobectomy in uncontrolled haemoptysis or localised disease

Question 27

complications of the prognosis

Answer 27

massive haemoptysis respiratory failure cor pulmonale ischaemic stroke

Question 28

describe the prognosis

Answer 28

irreversible condition, the typical disease course consists of periods of symptoms

Question 29

What is the primary ciliary dyskinesia?

Answer 29

motile cilia do not function properly- cannot move mucus in the respiratory tract that would lead to chronic respiratory infections