non cf bronchiectasis - rcp london

Non CF Bronchiectasis

Kate Williams

Respiratory Consultant

Kate.Williams@midyorks.nhs.uk

Aim

• Convince you why you need to know about bronchiectasis?

• Overview of investigations & principles of management of bronchiectasis.

• Know how to manage a simple exacerbation.

• Not talk for more than 40 minutes

Irreversibly damaged & dilated bronchi resulting in chronic airway inflammation & recurrent infections.

Bronchiectasis

The dilatation of the bronchi without being a very common affection, is, however, much less rare than I long conceived it to be (Laennec 1835)

Bronchiectasis is common

• Prevalence is approximately 1 in 1000.

• Recent UK & German studies have demonstrated increasing prevalence & hospitalisation rates.

• My patients with bronchiectasis experience:

• High rate of exacerbations

• Frequent hospital admissions

• Increased mortality

When should you suspect bronchiectasis?

• Chronic cough

• Chronic sputum production • Often large amounts

• Recurrent respiratory tract infections

• Dyspnoea

• Haemoptysis

• Colonisation with pseudomonas

• Coarse inspiratory & expiratory crackles

So always think about the cause

Bronchiectasis is an endpoint

Investigations

• HRCT “Gold Standard”

• FBC, ESR, CRP, U&E, LFTs

• Sputum culture

• 3 sputum samples for AAFB (smear & culture)

• Immunological screen & auto-antibodies

• Total IgE & Aspergillus screen

• PFTs

Further tests dependent on clinical suspicion eg ciliary investigations

Exclude cystic fibrosis

• All patients aged <40 presenting with bronchiectasis should have investigations for cystic fibrosis

• sweat chloride

• CFTR genetic mutation analysis

• Consider if aged >40 if:

• persistent isolation of Staphylococcus aureus

• features of malabsorption

• male primary infertility

• upper lobe bronchiectasis

CXR: low sensitivity, limited information Cylindrical bronchiectasis with tramline opacities

Cystic bronchiectasis

HRCT: Features of bronchiectasis Severity: although CT does not reliably correlate with QoL

Bronchial wall dilatation

Bronchial wall thickening & mucus plugging

Distribution

Investigating bronchiectasis does makes a

difference

• Pasteur at al 2000

• Cause identified 47% (70 / 150)

• Affected management 15%

• Li et al 2005 (Paediatric cohort)

• Cause identified 82% (111 / 136)

• Affected management 56%

• Shoemark et al 2007

• Cause identified 74% (122/165)

• Affected management 37%

Start with the underlying cause.

Treating bronchiectasis

Aims of Treatment

• Prevent progression of disease

• Maintain or improve lung function

• Decrease exacerbations

• Improve symptoms

• Improve quality of life

Increased volume & purulence of sputum, cough, breathlessness and/or systemic symptoms

Defining an Exacerbation

Always send a sputum sample Green is bad

Infections matter to the patient

Empirical Treatment

• Amoxicillin 1g tds [14/7] • Clarithromycin 500mg bd [14/7] • If colonised with P aeruginosa:

ciprofloxacin 500-750g bd

Modifying antibiotics

• Based on sputum culture or if

failure to improve. • Consider IVs if very unwell • If P. aeuruginosa use ≥1

antipseudomonal antibiotics eg β-lactamase & an aminoglycoside

Pseudomonas

• Associated with more severe

disease

• More rapid decline in FEV1

• Increased admissions

• Worse quality of life

• Reduced survival

• Consider eradication after 1st culture

BTS Guidelines

Nebulised Antibiotics

• Increased drug concentrations locally

• Reduce systemic adverse effects

• Can be done at home

• Use for chronic disease management or:

• Prophylaxis

• Eradication of pseudomonas

• Acute treatment

Any Questions?