Bronchiectasis

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Bronchiectasis\: chronic respiratory disease with permanent bronchial dilation due to irreversible bronchial wall damage.
Prevalence\: 3 per 1000 in the UK; 60% in patients >70 years; increased by 60% over the last 20 years.
Aetiology\:
Post-infectious\: recurrent childhood LRTIs, TB, ABPA.
Pulmonary disease\: COPD, asthma.
Congenital\: cystic
Connective tissue disease\: RA, SLE, sarcoidosis.
Idiopathic\: 40% of cases.
Risk factors\: age >70, female gender, smoking history.
Symptoms\: daily cough with copious mucopurulent sputum, exertional/resting dyspnoea, fatigue, rhinosinusitis symptoms.
Examination\:
Di
sinusitis (normal radiology).
Investigations\:
Bedside\: pulse oximetry, sputum culture, lung function tests, echocardiogram.
Laboratory\: FBC, CRP, autoimmune screen, speci
Imaging\: chest X-ray, high-resolution CT chest, bronchoscopy.
Management\:
Conservative\: pulmonary rehab, smoking cessation, annual in
Medical\: mucoactive agents, long-term antibiotics, bronchodilators, CFTR modulators, long-term oxygen therapy.
Surgical\: lung resection, lung transplant.
Complications\:
Disease-related\: respiratory failure, massive haemoptysis, anxiety and depression.
Treatment-related\: long QT syndrome, hearing loss (macrolides), transplant complications.
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Introduction

Bronchiectasis is a chronic respiratory disease characterised by permanent bronchial dilation, due to irreversible
damage to the bronchial wall.
1
In the UK, the prevalence of bronchiectasis is 3 per 1000, and 60% of diagnoses are in patients over the age of 70. The
prevalence of bronchiectasis in the UK has increased by 60% over the last 20 years.
2,3
Patients with mild bronchiectasis often have normal life expectancies. The mortality risk increases in patients with more
frequent exacerbations and worse lung function.
4Aetiology
Pathophysiology
The pathogenesis of bronchiectasis is poorly understood. An initial insult to the bronchi (e.g. infection) results in immune
cells being recruited to the bronchi. These immune cells secrete cytokines and proteases, leading to in
bronchi.
This in
In most people, this bronchial dilation is reversible after the resolution of the initial insult to the bronchi. However, in patients
with bronchiectasis, several factors prevent the bronchial dilation from reversing (e.g. impaired mucociliary clearance and
dysregulated immunity).
7
Dilated bronchi are predisposed to persistent microbial colonisation, as mucus traps in the dilated bronchi (
Therefore, bronchiectasis patients get caught in a vicious cycle, whereby their airways are colonised by micro-organisms,
which increases bronchial in
colonisation.
8
Figure 1.A normal airway compared to a bronchiectatic airway.
9
Causes of bronchiectasis
There is a wide range of possible underlying causes of bronchiectasis, which are summarised below.
Post-infectious (most common cause)\:
Recurrent childhood lower respiratory tract infections (e.g. in
Pulmonary tuberculosis
Allergic bronchopulmonary aspergillosis (ABPA)
Pulmonary disease\:
Chronic obstructive pulmonary disease
Asthma (especially in patients with frequent exacerbations and neutrophilic asthma)
5
Congenital\:
Cystic
Primary ciliary dyskinesia
Alpha-1 antitrypsin de
Connective tissue disease\:Rheumatoid arthritis
Systemic lupus erythematosus (SLE)
Sarcoidosis
Idiopathic (40% of bronchiectasis)
6

Risk factors

Risk factors for bronchiectasis include\:
Age (>70 years)
Female gender
Smoking history

Clinical features

History
Typical symptoms of bronchiectasis include\:
10
Daily cough, producing copious amounts of mucopurulent sputum (haemoptysis is present in 50% of patients)
Exertional dyspnoea, which may progress to resting dyspnoea
Fatigue
impairment
Other important areas to cover in the history include\:
Rhinosinusitis symptoms (such as nasal discharge, nasal obstruction and facial pressure)\: due to underlying mucociliary
History of childhood lower respiratory tract infections\: you may forget to ask this if the patient is elderly
Family history\: ask about congenital conditions (such as cystic
arthritis)
Smoking history\: quantify in pack-years (1 pack-year = smoking 20 cigarettes a day for a year)
Clinical examination
A full respiratory examination should be performed in suspected cases of bronchiectasis.
Typical clinical
Finger clubbing\: due to increased secretion of growth factors, leading to increased growth of extracellular matrix in the
nails
11
Course crepitations, present in expiration and inspiration\: caused by sudden opening and closing of the airways
12Rhonchi (low-pitched noises, which sound like snoring)\: caused by the movement of secretions in the large airways
High-pitched inspiratory squeaks and pops\: unclear aetiologyFigure 2. Finger clubbing.
13

Di

The main presenting complaint of bronchiectasis (chronic productive cough) has important di
Table 1 outlines these di
Table 1. Di
DiChronic obstructive
pulmonary disease
Asthma
Pneumonia
Chronic sinusitis
Features di
Reduced breath sounds
Absence of high-pitched inspiratory squeaks
CT chest may be normal
Diurnal variation in symptoms and peak
History of atopy
Lung function tests\: bronchodilator reversibility
More acute presentation (over days, rather than
months or years)
Chest X-ray\: consolidation
Vesicular breath sounds
Radiological investigations\: normal

Investigations

Bedside investigationsRelevant bedside investigations include\:Pulse oximetry\: aim for 94-98% initially, but the target saturations may be reduced in advanced disease (this is a risk-
bene
Sputum culture\: common organisms isolated include P s e u d o m o n a s a e r u g i n o s a and H a e m o p h i l u s i n
Pseudomonas is particularly common, as it forms bio
14
Lung function tests\: typically show an obstructive pattern (FEV1/FVC ratio \< 70%), but may be normal
Echocardiogram\: bronchiectasis may impair ventricular function and lead to pulmonary hypertension
15
Laboratory investigations
Relevant laboratory investigations include\:
Full blood count\: may show elevated white blood cell count, including neutrophilia
CRP\: may be elevated during acute exacerbations
Autoimmune screen (if suspecting an autoimmune condition)\: includes anti-CCP, ANA and ANCA
Speci
Genetic testing (done in specialist units)\: to diagnose congenital disorders, such as cystic
dyskinesia
Imaging
Relevant imaging investigations include\:
Chest X-ray\: required to exclude other pathologies. Chest X-ray may be normal in mild bronchiectasis. Signs in severe
disease include tram lines and ring shadows.
High-resolution CT chest (
thickening.
16
Bronchoscopy (
or an endobronchial lesion.
17
Figure 3. CT Chest showing bronchiectatic airways (white and black arrows).
18

Management

Conservative management
Conservative management options for bronchiectasis include\:Pulmonary rehabilitation\: refer the patient to a respiratory physiotherapist, who will teach the patient airway clearance
techniques (which o
Smoking cessation\: see the Geeky Medics guide on smoking cessation
Annual in
Medical management
Medical management options for bronchiectasis include\:
Mucoactive agents (e.g. nebulised saline and carbocisteine)\: aid the clearance of sputum, for patients who have di
expectorating sputum (such as frail, elderly patients)
17
Long-term antibiotics (e.g. azithromycin three times a week)\: may be used in patients who have three or more
exacerbations per year, after consultation with a respiratory specialist
1
Bronchodilators\: o
Speci
Long-term oxygen therapy\: if saturations on room air are \<88% or PaO2 on room air is \<7.3kPa
Surgical management
Surgical management options for bronchiectasis include\:
Lung resection\: for localised bronchiectasis, not controlled by optimum medical management
Lung transplant (
17
Figure 5.Clamshell incision scar, resulting from a bilateral lung transplant.
20

Complications

Disease-related complications of bronchiectasis include\:
Respiratory failure\: due to failure of gas exchange in the lungs
Massive haemoptysis (>250ml per day)\: often due to rupture of a bronchial artery into a bronchus
21
Anxiety and depression\: due to impaired quality-of-life
Treatment-related complications of bronchiectasis include\:
Macrolides\: long QT syndrome, tinnitus and hearing loss
Lung transplant\: immediate complications (e.g. blood loss), early complications (e.g. transplant rejection) and late
complications (e.g. post-transplantation lymphoproliferative disorder)Feedback
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Reviewer

Dr Neeraj Shah
Respiratory medicine registrar

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