Bronchiectasis - definition
Bronchiectasis is the permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall. It is often caused as a consequence of recurrent and/or severe infections secondary to an underlying disorder.
Bronchiectasis - aetiology
- Post-infectious: viruses (i.e., measles, influenza, pertussis), Mycobacteria or severe bacterial pneumonia
- Immunodeficiency: Immunoglobulin deficiency, HIV
- Genetic: CF, ciliary dyskinesia +/- Kartagener’s syndrome, A1ATd, Ehlers-Danlos, Marfan’s
- Aspiration or inhalation injury
- Connective tissue disorders: RA, Sjogren’s
- Inflammatory bowel diseases: UC, Crohn’s
(all of these are risk factors)
Pathophys
- Persistent airway inflammation
- Bronchial wall oedema and increased mucus
- Neutrophils, T lymphocytes, +other are recruited
- Release inflammatory cytokines, proteases, and reactive oxygen mediators
- Progressive destruction of the airways
- Decreased ability to clear secretions
- Microbes collect, further inflammation and dilation
Clinical features
- Cough - may be a/w large amounts of purulent sputum and, less commonly, haemoptysis
- dyspnoea esp on exertion
- fever +/- fatigue, malaise
- crackles, high-pitched inspiratory squeaks and rhonchi
- wheeze
Investigations
- spirometry: may be normal or obstructive
- sputum culture
- CXR
- Post-bronchodilator spirometry
- gold standard: High-resolution CT (HRCT)
- PFTs: reduced FEV1, elevated RV/TLC
consider testing for CF, serum A1AT etc
CXR - findings
- May be normal
- Ring opacities – dilated end of bronchi
- Tram-tracks - dilated airways seen in a horizontal orientation
- Fluid-filled cysts or bronchocoeles
- may show obscured hemidiaphragm, tubular or ovoid opacities
- coarse patches
HRCT - findings
- Signet ring sign – resembles a signet (or pearl) ring, which is an opacity representing a dilated bronchus in cross section and a smaller adjacent opacity representing its pulmonary artery
- Tram-tracks – dilated airways seen in a horizontal orientation
- Lack of tapering of airways, varicose constrictions
- Mucus impaction, cysts
- Mosaicism
Bronchiectasis - Management
- conservative: check BMI (encourage weight loss if needed), smoking cessation, pulmonary rehabilitation (physical training e.g. inspiratory muscle training; breathing techniques)
- physio: airway clearance therapy includes postural drainage, percussion, vibration. The primary goal is bronchopulmonary hygiene.
- treatment of infection – 10-14 day course of Abx
- Influenza/pneumococcal vaccine
- Test sputum culture for unusual/colonising organisms
- Consider checking vitamin D levels
Which antibiotic is prescribed for Pseudomonas aeruginosa?
- Only orally active antimicrobial is Ciprofloxacin
- IV options include Tazocin (Piperacillin and Tazobactam), Ceftazidime
Exacerbation - features
- worsening cough
- change in sputum colour and an increase in sputum volume
- increasing breathlessness
- haemoptysis may become massive (>250 mL/day), which warrants hospital admission
- may have persistent pleuritic chest pain
Criteria for hospital admission (general)
Any signs suggesting a more serious illness or condition (such as cardiorespiratory failure or sepsis):
- Cyanosis
- Confusion
- RR> 25
- Marked breathlessness, rapid respiration, or laboured breathing.
- Peripheral oedema
- T of 38°C or more.
Exacerbation - management
- Preview previous sputum culture results and most recent course of antibiotics given
- Send more sputum for culture
- Choose Abx (in line with local guidance)
Amoxicillin/clarithromycin/doxycycline oral
Ciprofloxacin if pseudomonas aeruginosa - Total course 10-14 days
- May need to consider outpatient IV antibiotics
- Chest physiotherapy
- Prescribe a SABA (such as salbutamol) if necessary
What are the most commonly involved pathogens?
Haemophilus influenzae (most common)
Pseudomonas aeruginosa
Klebsiella spp.
Streptococcus pneumoniae
