Bronchiectasis.PLOUGHING 24

Bronchiectasis
Possible scenarios: Fatigue, shortness of breath, difficulty in

breathing, reduced exercise tolerance, productive cough.
Examining bronchiectasis: Look for and examine sputum pots (which

may include haemoptysis), clubbing of the fingers (not always
present), get the patient to cough on hearing crackles as the character
should change and there may be an associated wheeze. The crackles
can occur at any point in the respiratory cycle. Sometimes inspiratory
clicks and crackles can be heard with the unaided ear. You need to ask
yourself four questions…
Which part of the lung is affected?
Is there a possible underlying cause identified examining peripherally?
Are there any signs of treatment such as steroids or long term

antibiotics? A line in the arm is likely a PICC line, a line under the
clavicle is likely a Hickmann line, and these may have buttoned
endings called ports at their ends.
Are there any signs of complications such as cor pulmonale?
Classic cases:
Rheumatoid arthritis: Symmetrical deforming polyarthropathy mainly

affecting the small joints of the hands which spares the distal
interphalangeal joints (DIPJs) and is associated with ulnar deviation
and dorsal subluxation of metacarpophalangeal joints (MCPJs), and
nodules on the extensor aspects of the forearm. Specific issues to
address – presence of immunosuppression, presence of infection,
urine dip for proteinuria.
Cystic fibrosis: Slim and short, pale, diabetic finger prick marks,
clubbing, scars from previous or current long lines for antibiotics.
Offer the resp investigations but also sweat test for sweat sodium and
chloride >60mmol/L, faecal elastase, abdo USS for liver, CT sinuses,
DEXA scans, family counselling and testing.
Kartagener’s Syndrome: Slim and short, pale, clubbing, scars from

previous or current long lines for antibiotics; dextrocardia clinches the
diagnosis (think about this when you’ve decided in your head that this
might be bronchiectasis and you’re checking for chest expansion – can
you feel the apex beat on the right side?). If dextrocardia is found
then proceed to percuss and palpate for liver dullness on the left
which would indicate situs inversus.
Yellow nail syndrome: Yellow, slow growing nails with some degree of
onycholysis and very curved, and features of a pleural effusion or
bronchiectasis on the chest, and lymphoedema elsewhere.
Associated signs:
Cor pulmonale: Raised JVP, left parasternal/right ventricular heave,

loud P2, split second heart sound, peripheral oedema.
Respiratory failure: Flap of outstretched hands, drowsy, cyanosed.
Infection: Antibiotics, fever, bronchial breathing.

Scars: Chest drains for empyema.
Differential diagnosis:
Congenital or acquired.
Idiopathic.
Inherited: Cystic fibrosis, Kartagener’s syndrome, primary ciliary

dyskinesia, yellow nail syndrome.
Infection: Tuberculosis, aspiration, childhood infections including

measles and pertussis.
Post-obstruction: Foreign body aspiration.
Hypersensitivity: ABPA, rheumatoid arthritis, inflammatory bowel

disease associated.
Immunodeficiency: Hypogammaglobulinaemia, AIDS.
Differential of clubbing with coarse crackles includes malignancy,

supparative lung infections, and pulmonary fibrosis with co-existent
infection.
Further investigations:
Bedside tests: PEFR, ECG (right heart strain), ABG (hypoxia,
respiratory failure, acidosis), obtain a sputum sample (send for MCS
and AFBs; cytology to r/o a neoplastic process), urine dipstick (for
proteinuria suggestive of amyloidosis).
Bloods: FBC (anaemia, polycythaemia, high white cell counts),

CRP/ESR (markers of inflammation), immunoglobulins including total
IgE (immunodeficiency, ABPA), aspergillus serology and functional
antibody tests to tetanus, pneumococcus and haemophilus (functional
immunodeficiency); if under 40 with upper lobe bronchiectasis and no
cause found test for CF with sweat test and genetic screening.
Consider special tests such as autoimmune screen including ANA, RF,
and ACE.
Imaging: CXR (tramlines, ring shadows), high resolution CT (signet ring

sign, localised or generalised).
Special tests: Spirometry (often obstructive, normal or mixed pattern),

bronchoscopy (to obtain microbiological or tissue specimens, exclude
obstructive lesion if localised).
Referral:
Probably respiratory, consider if inpatient assessment is required.
Management:
Conservative: Smoking cessation, postural drainage (assisted by
mucolytics and nebulised saline), active cycle breathing,
pneumococcal and annual influenza vaccinations.
Medical: Treat intercurrent infections, treat colonising infections

(nebulised, oral or parenteral), inhalers, anti-inflammatories (3 times
a week azithromycin), LTOT.
Surgical: Drainage or removal of localised bronchiectasis or abscess,

bronchial artery embolisation if massive haemoptysis.
Questions:
What is bronchiectasis? A permanent, pathological dilatation of the

bronchi and bronchioles.
What are the complications of bronchiectasis? Infective

exacerbations, empyemas, abscesses, cerebral abcesses,
pneumothoraces, cor pulmonale, and amyloidosis.
What infections are seen in patients with bronchiectasis?

Staphylococcus aureus, haemophilus influenzae, pseudomonas,
pneumococcus, klebseilla, aspergillus.
What is the mode of inheritance of Kartagener’s Syndrome?

Autosomal recessive – KS encompasses dextrocardia, situs inversus,
bronchiectasis and sinusitis with primary ciliary dyskinesia.
*Example presentation: My presumed diagnosis in this young female

is bronchiectasis as evidenced by the presence of clubbing and coarse
inspiratory and expiratory crepitations that alter with coughing and
are associated with inspiratory clicks. She is slim and has what looks
like a Hickman line in situ. I think the most likely diagnosis is Cystic
Fibrosis or Primary Ciliary Dyskinesia but my differential diagnosis is
idiopathic, infection related including childhood infections and
tuberculosis, hypersensitivity related such as ABPA, rheumatoid and
inflammatory bowel disease associated, and immune deficiency states
such as hypogammaglobulinaemia and HIV. There is no evidence of
respiratory failure or cor pulmonale on examination today and she is
not Cushingoid. To investigate further I would like to take a history,
get a full set of bedside observations, examine the sputum pots and
send them for MCS and cytology, and request spirometry to look for a
restrictive or obstructive lung defect wit reduced gas transfer. On her
blood tests I would be particularly interested in her autoimmune
screen, viral screen, immunoglobulins and functional antibody tests to
tetanus, pneumococcus and haemophilus, and consider sweat testing
for CF. The imaging modality of choice would be a high resolution CT
scan of the chest for signet ring sign and airway dilatation. She may
require bronchoalveolar lavage or biopsy. I would counsel the patient
on the diagnosis and signpost to appropriate support groups, advise
smoking cessation, pneumococcal and influenza vaccination, and refer
to respiratory physiotherapy for advice on postural drainage and
active cycle breathing. I would treat any underlying diseases, offer
inhalers and anti-inflammatories, treat intercurrent or colonising
infections, and consider the need for long term oxygen therapy. Some
patients are eligible for single or double lung or combined lung-heart
transplants.

Bronchiectasis.PLOUGHING 24

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Bronchiectasis

Possible scenarios: Fatigue, shortness of breath, difficulty in

Examining bronchiectasis: Look for and examine sputum pots (which

Which part of the lung is affected?

Is there a possible underlying cause identified examining peripherally?

Are there any signs of treatment such as steroids or long term

Are there any signs of complications such as cor pulmonale?

Rheumatoid arthritis: Symmetrical deforming polyarthropathy mainly

Kartagener’s Syndrome: Slim and short, pale, clubbing, scars from

Cor pulmonale: Raised JVP, left parasternal/right ventricular heave,

Respiratory failure: Flap of outstretched hands, drowsy, cyanosed.

Infection: Antibiotics, fever, bronchial breathing.

Inherited: Cystic fibrosis, Kartagener’s syndrome, primary ciliary

Infection: Tuberculosis, aspiration, childhood infections including

Post-obstruction: Foreign body aspiration.

Hypersensitivity: ABPA, rheumatoid arthritis, inflammatory bowel

Immunodeficiency: Hypogammaglobulinaemia, AIDS.

Differential of clubbing with coarse crackles includes malignancy,

Bloods: FBC (anaemia, polycythaemia, high white cell counts),

Imaging: CXR (tramlines, ring shadows), high resolution CT (signet ring

Special tests: Spirometry (often obstructive, normal or mixed pattern),

Probably respiratory, consider if inpatient assessment is required.

Medical: Treat intercurrent infections, treat colonising infections

Surgical: Drainage or removal of localised bronchiectasis or abscess,

What is bronchiectasis? A permanent, pathological dilatation of the

What are the complications of bronchiectasis? Infective

What infections are seen in patients with bronchiectasis?

What is the mode of inheritance of Kartagener’s Syndrome?

*Example presentation: My presumed diagnosis in this young female

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