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Bronchiectasis

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Bronchiectasis is a chronic destructive infective inflammatory disorder of airways. Approximately 50% of cases are idiopathic, with no identifiable underlying cause.

About

Bronchiectasis is a chronic, debilitating condition characterized by abnormal and permanent dilatation of the airways.
Patients experience persistent cough, excessive sputum production, and frequent respiratory infections.
Despite advances in diagnosis and management, bronchiectasis continues to cause significant morbidity and negatively impacts quality of life.

Pathology

Bronchiectasis involves permanent abnormal dilatation and thickening of the bronchi due to chronic infection and inflammation.
Damage to the muscle and elastic layers of the bronchial walls leads to airway distortion and impaired mucus clearance.
Types include:
- Cylindrical Bronchiectasis: Uniform, tube-like dilatation of the airways.
- Saccular Bronchiectasis: Airways form balloon-like dilatations, often with more severe damage.
- Varicose Bronchiectasis: Irregular dilatation with a beaded appearance.
Pathological changes include ulceration, fibrosis, and squamous metaplasia of the bronchial epithelium.
Chronic infections, mucus plugging, and impaired mucociliary clearance contribute to a cycle of inflammation and damage.

Causes

Cause	Description	Comments
Infections	Chronic or recurrent infections, such as TB, pneumonia, or whooping cough.	Infections cause persistent inflammation, leading to airway damage and dilation.
Cystic Fibrosis (CF)	Genetic disorder leading to thick, sticky mucus in the lungs, causing chronic infections and bronchiectasis.	Most common genetic cause; 1 in 25 people in the UK carry a CF gene mutation.
Primary Ciliary Dyskinesia (PCD)	Impaired ciliary function prevents effective mucus clearance from the respiratory tract.	Recurrent infections lead to airway damage over time.
Immune Deficiencies	Conditions like common variable immunodeficiency (CVID) or HIV/AIDS increase susceptibility to infections.	Frequent and severe infections lead to bronchiectasis.
Allergic Bronchopulmonary Aspergillosis (ABPA)	Allergic reaction to the fungus Aspergillus causing airway inflammation and damage.	Common in patients with asthma or CF.
Autoimmune Diseases	Conditions like rheumatoid arthritis or inflammatory bowel disease can cause inflammation in the lungs.	Can lead to airway damage and subsequent bronchiectasis.
Post-Obstructive Bronchiectasis	Obstruction of the airway by tumors, foreign bodies, or enlarged lymph nodes.	Common cause globally is tuberculosis.
Idiopathic	No identifiable underlying cause.	Accounts for a significant proportion of cases, particularly in older adults.

Clinical Features

Persistent cough with large volumes of sputum, often foul-smelling.
Recurrent respiratory infections, including pneumonia, which can worsen airway damage.
Sputum characteristics: mucoid, mucopurulent, or purulent; measure volume over 24 hours for assessment.
Dyspnoea on exertion or at rest, depending on severity.
Haemoptysis, ranging from streaks of blood to massive, life-threatening bleeding.
Finger clubbing may be present in advanced or longstanding cases.
Physical examination may reveal coarse crackles over affected areas, wheezing, or reduced breath sounds.
Symptoms of chronic fatigue and weight loss may occur due to the high metabolic demand of chronic inflammation.

Investigations

Test	Description	Notes
Blood Tests	Raised WCC, ESR, CRP may indicate active infection or inflammation.	Monitor inflammatory markers during exacerbations.
CXR	May show tram tracks, ring shadows, or honeycomb patterns in advanced disease.	Less sensitive than HRCT for diagnosing bronchiectasis.
High-Resolution CT (HRCT) Scan	Gold standard for diagnosis; shows airway dilation, wall thickening, mucus plugging, and tree-in-bud pattern.	Allows assessment of disease extent and severity.
Spirometry	Measures lung function, specifically FEV1 and FVC to assess obstruction.	Used to monitor lung function over time and assess response to treatment.
Sputum Culture	Identifies bacteria, fungi, and mycobacteria.	Guides antibiotic choice for exacerbations and chronic suppression therapy.
Aspergillus Precipitins	Tests for antibodies against Aspergillus in patients with suspected ABPA.	Positive in ABPA and may require antifungal therapy.
Bronchoscopy	Allows direct visualization of airways and sampling of secretions.	Useful in cases of suspected malignancy, foreign body, or localized bronchiectasis.
Genetic Testing	Tests for CFTR gene mutations or primary ciliary dyskinesia.	Indicated in patients with early-onset disease or family history.
Pulmonary Function Testing	Includes lung volumes and diffusion capacity (DLCO).	Assesses overall lung function and aids in management decisions.

Management

Management Option	Description	Comments
Airway Clearance Techniques	Chest physiotherapy, postural drainage, and oscillating positive expiratory pressure (PEP) devices.	Essential to reduce mucus build-up and prevent infections.
Bronchodilators	Short-acting beta agonists (SABA) and long-acting beta agonists (LABA) to open airways.	Beneficial in patients with coexisting asthma or COPD.
Inhaled Corticosteroids (ICS)	May reduce inflammation in patients with asthma overlap or frequent exacerbations.	Not typically first-line for bronchiectasis alone.
Long-term Antibiotic Therapy	For patients with frequent exacerbations (≥3/year) or chronic Pseudomonas infection.	Options include macrolides (azithromycin) or inhaled antibiotics (tobramycin, colistin).
Vaccinations	Annual influenza and pneumococcal vaccinations.	Reduces risk of infections that can exacerbate bronchiectasis.
Surgery	Localized disease or significant hemoptysis not responsive to medical management.	Resection of the affected lung area may be needed.
Pulmonary Rehabilitation	Exercise, education, and support to improve physical conditioning.	Improves quality of life and reduces symptoms.

Complications

Frequent respiratory infections, leading to lung function decline.
Respiratory failure in advanced cases due to loss of effective lung function.
Massive haemoptysis, which can be life-threatening.
Pulmonary hypertension due to chronic hypoxia and lung changes.
Right heart failure (cor pulmonale) in severe disease.
Systemic amyloidosis due to chronic inflammation.

Prognosis

Depends on the underlying cause, frequency of exacerbations, and presence of chronic infections.
Patients with chronic Pseudomonas infection generally have a poorer prognosis.
Early diagnosis and adherence to airway clearance techniques and infection management improve outcomes.

References

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