Related Subjects:
Asthma
|Acute Severe Asthma
|Exacerbation of COPD
|Pulmonary Embolism
|Cardiogenic Pulmonary Oedema
|Pneumothorax
|Tension Pneumothorax
|Respiratory (Chest) infections Pneumonia
|Fat embolism
|Hyperventilation Syndrome
|ARDS
|Respiratory Failure
|Diabetic Ketoacidosis
Chronic destructive infective inflammatory disorder of airways. 50% are idiopathic
About
- Bronchiectasis is a chronic, debilitating condition
- Persistent cough, excessive sputum production and recurrent chest infections.
- Pathologically there is abnormal and permanent dilatation of the airways
Pathology
- Permanent abnormal destructive dilatation of the bronchi with damage to muscle and elastic layers
- Pathologically classified as saccular, tubular and fusiform
- There is also Ulceration, fibrosis and squamous metaplasia
- May be generalised but can indeed be localized causing abnormal dilatation of the airways
- Chronic infection, pus formation perhaps distal to some obstructive lesion such as a tumour, foreign body or enlarged lymph nodes
Causes
- 50% are idiopathic and the rest mostly post-infective
- Worldwide the commonest cause is Tuberculosis
- Western world commonest cause is Cystic fibrosis
- Pertussis and viral infections
- Rheumatoid disease, Allergic bronchopulmonary disease
- Ciliary dysfunction - Kartagener's syndrome - Bronchiectasis + Situs inversus
- Immunodeficiency syndromes
- Young's syndrome - Bronchiectasis + Male infertility (palpable enlarged epididymis)
- Gastro-oesophageal reflux disease
Clinical
- Cough with large amounts of expectorant sputum or even halitosis
- Acute on chronic infective episodes
- Sputum is mucoid, mucopurulent or purulent - measure volume over 24 hours
- Finger clubbing, wheeze copious sputum and long term amyloidosis
- Patients may have tiredness and malaise
- Massive haemoptysis may be seen
Investigations
- ? WCC ? ESR ? CRP suggests acute infection
- Low IgG levels in immunodeficiency or low IgA
- CXR - tram lines and signet ring signs
- HRCT - imaging of choice shows cystic or varicose dilatations often affecting multiple lobes. Bronchial wall thickening, mucus plugging and segmental and subsegmental collapse can be seen.
- Spirometry - often an obstructive pattern but a restrictive pattern may be seen
- Saccharin test may be useful if Ciliary dysfunction syndromes such as Kartagener's syndrome or primary Ciliary dyskinesia which are then confirmed using electron microscopy
- Aspergillus precipitins
- Sweat test if Cystic fibrosis considered - see the section on CF
- Sputum cultures - Haemophilus influenzae, Streptococcus pneumonia, Staphylococcus aureus, Moraxella catarrhalis, Pseudomonas aeruginosa (poorer prognosis)
Management
- Postural drainage can be taught and followed by the patient usually 20 minutes twice daily.
- Good nutrition is also key and dietetics help is useful where nutritional support is needed.
- Ensure patient has Influenza and Pneumococcal vaccination
- Antibiotics when needed for S pneumoniae and H. influenzae infections
- If there is suspicion of Pseudomonas infection then this will require usually IV antibiotics
- Smoking cessation is vitally important
- Inhaled hyperosmolar agents to aid expectoration (7% hypertonic saline) improve expectoration
- Inhaled mucolytic e.g. recombinant human DNAse though non evidence-based
- Inhaled bronchodilators may be tried to improve dyspnoea
- Inhaled steroids may be tried to improve dyspnoea
- Long term antibiotics have had mixed results
- Surgery in individual selected cases with localised disease which can be resected
- Rarely embolisation for severe haemoptysis is attempted