Bronchiectasis

Bronchiectasis is a chronic respiratory condition characterised by the irreversible widening and scarring of the bronchi, leading to recurrent infections and a decline in lung function. While relatively rare, bronchiectasis can be a debilitating condition that severely impacts a patient’s quality of life. It is important to recognise the signs and symptoms of bronchiectasis, as early diagnosis and appropriate management can significantly improve patient outcomes. In this article, we will discuss the causes, clinical presentation, diagnostic criteria, and treatment options for bronchiectasis, providing you with the tools necessary to effectively diagnose and manage this challenging respiratory condition.

image

Source: https://www.myupchar.com/en/disease/bronchiectasis (Creative Commons License)

What is bronchiectasis?

Bronchiectasis is a clinical syndrome comprised of:

  1. Abnormal and increased production of mucopurulent sputum from bronchial walls;
  2. Abnormal bronchial wall dilatation (of proximal and medium-sized bronchi), irreversible bronchial wall destruction and transmural inflammation which is demonstrable radiologically.

The consequence of these pathological changes is:

  • Impaired clearance of secretions;
  • Bacterial colonisation of secretions; and
  • Clinical presentation with recurrent infection and lung damage

What are some key clinical features of bronchiectasis?

  • Clubbing
  • Productive cough with copious sputum production
  • Coarse crepitations (inspiratory and expiratory) – distribution related to causes of bronchiectasis and altering with cough
  • Wheeze
  • Peripheral signs of inflammatory disease e.g. rheumatoid arthritis, systemic lupus erythematosus
  • Nicotine staining (30% of patients with COPD have bronchiectasis)
  • Signs of pulmonary hypertension – right ventricular heave, raised JVP, peripheral oedema
  • Lymphadenopathy – present in sarcoidosis and tuberculosis, but also cancer

Example Case

A 22-year old boy has a history of recurrent chest infections. He had problems with a productive cough early in his life. Over the past few years, he has been considered to be quite a “chesty child” and often spent 4-5 weeks away from university in hospital.

On multiple occasions, Haemophilus influenzae was grown on sputum culture. During his last admission, culture grew Pseudomonas sp. There is no family history of respiratory problems. He has no other symptoms aside from an irregular bowel habit. On examination, he has coarse crepitations over the upper zones of his lungs and has a low BMI.

What would be your suspected diagnosis, and what investigations would you perform to confirm this?

image 2

Macroscopic pathology of bronchiectasis. Source: BruceBlaus (Creative Commons License)

Causes

In a patient with clinico-radiological features of bronchiectasis, one should obtain a careful history to elicit the potential underlying cause of bronchiectasis.

The likely cause will alter depending on the age that the patient presents. 

This may have already been established, but if not:

  • Childhood respiratory infection – measles, pertussis and TB
  • Genetic muco-ciliary clearance defects – cystic fibrosis, Kartagener’s syndrome, Young syndrome, immotile cilia syndrome
  • Immunodeficiency – congenital and acquired hypogammaglobulinemia, AIDS
  • Allergic Bronchopulmonary Aspergillosis (exposure to fungal mold)
  • Autoimmune disease – rheumatoid arthritis, Sjogren’s syndrome, Inflammatory Bowel Disease
  • Bronchial obstruction – Foreign body, chronic aspiration (e.g. patients with alcohol dependence syndrome, gastro-oesophageal reflux disease), endobronchial tumour, lymphadenopathy (tuberculosis/sarcoidosis/malignancy), granulomatous diseases
  • Congenital anatomical defects – Yellow Nail syndrome,Swyer-James syndrome, William-Campbell syndrome
  • Idiopathic

Important differential diagnoses to consider in a patient with clubbing, coarse crepitations and cough

  • Bronchiectasis
  • Lung abscess
  • Pulmonary fibrosis

Key investigations

Bedside
  • Pulse oximetry
  • Arterial blood gas – for evidence of hypoxaemia or hypercapnic respiratory failure
  • Sputum MC&S for general, fungal & mycobacterial culture – patients with bronchiectasis are at high risk of bacterial colonisation by potentially pathogenic organisms, microbiological identification will help target treatment ; regular samples to monitor for Pseudomonas colonisation
Imaging
  • Chest x-ray – Dilated and thickened airways (tramlines and ring shadows which may contain air fluid levels)
  • HRCT – Gold-standard for diagnosis; note radiological severity ≠ clinical severity
    • Signet-ring sign = bronchial diameter >1.5 x adjacent vessel diameter
    • Lack of bronchial tapering
    • Reid classification = cylindrical (most advanced), varicose and saccular (cystic) bronchiectasis
    • Distribution may be indicative of cause (e.g. ABPA, cystic fibrosis, idiopathic)
image1
Signet ring sign with short arrow, “string of pearls” sign long arrow. Indicative of central bronchiectasis.
Source
: https://www.cureus.com/articles/19187-diagnosing-allergic-bronchopulmonary-aspergillosis-a-review. (Creative Commons License)
Special tests
  • Spirometry: obstructive spirometry (reduced FEV1/FVC)and not always reversible; restrictive if severe advanced disease due to scarring or atelectasis, or due to underlying pulmonary fibrosis
  • Lung function tests: usually normal 
  • Echocardiography: to look for signs of pulmonary hypertension
  • Cause-specific bloods/tests:
    • Immunodeficiency: immunoglobulins,HIV serology 
    • Aspergillus: aspergillus serology (Aspergillus precipitins), fungal cultures, skin prick and RAST tests, galactomannan
    • Cystic fibrosis: sweat test, genetic testing
    • Rheumatoid arthritis/connective tissue diseases: Rheumatoid factor, anti-CCP, autoantibodies (ANA, ENA, anti-dsDNA, C3, C4, lupus anticoagulant, ANCA, anti-Ro, anti-La)

Distribution by cause

  • Allergic bronchopulmonary aspergillosis -> upper lobes involving central bronchi
  • Tuberculosis/fungal infection ->  upper lobes
  • Bronchial obstruction/recurrent aspiration -> right middle lobe
  • Infection -> Lower lobes, right middle lobe and lingula

Common pathogens implicated in bronchiectasis

  • Staphylococcus aureus
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Streptococcus pneumoniae
  • Klebsiella pneumoniae
  • Aspergillus sp.
  • Note that non-tuberculous mycobacteria and Burkholderia cenocapacia are associated with poor prognosis, and in some transplant centres Burkholderia sp. may be an absolute contraindication to transplantation

Complications

  • Infection
  • Pneumothorax
  • Massive haemoptysis
  • Empyema
  • Frequent hospital admissions
  • Collapsed lung
  • Respiratory failure
  • Pulmonary hypertension
  • Death

Treatment

Treatment of this condition is multidisciplinary, and involves a specialist respiratory physician, specialist nurses, physiotherapists and dietitians. 

The goals of treatment include rapid treatment and prevention of infections, optimum bronchial clearance and optimum quality-of-life for the patient. 

General measures
  • Smoking cessation
  • Nutrition e.g. oral nutritional supplementation, Creon enzymatic supplementation may be required in cystic fibrosis due to concurrent pancreatic insufficiency; in some cases, long-term enteral feeding via a PEG may be required
  • Flu/pneumococcal/COVID vaccinations
  • Confirm immunity to measles/pertussis/rubella
  • Postural drainage and chest physiotherapy – percussion and vibration, flutter and pneumatic compression devices, nebulised saline and mucolytics. Please note the first trial of hypertonic saline nebulisers should be observed due to the risk of acute bronchospasm. Offer active cycle of breathing techniques or oscillating positive expiratory pressure.
  • Sputum MC&S – routine bacteriology and fungal culture, 3 x sputum samples for Mycobacterial Culture 
image2
Nebuliser.
Source: Marco Verch. (Creative Commons License)
Medical management
  • Prompt treatment of infective exacerbations with antibiotics – long-term, parenteral or nebulisers depending on clinical situation; 2-week course to be guided by sputum MC&S 
  • Consider long-term antibiotics in patients with bronchiectasis who experience 3 or more exacerbations per year; central line (e.g. Portacath) or midline may be required for patients requiring frequent courses of intravenous antibiotics – under specialist respiratory supervision/guidance
  • Microbiology input with regards to antimicrobial treatment and suitability for Outpatient Parental Antimicrobial Therapy (OPAT) 
  • Nebulised colistin for Pseudomonas colonisation and prevention of exacerbations – under specialist respiratory supervision/guidance
  • Pseudomonas eradication with ciprofloxacin/IV piperacillin 
  • Oxygen supplementation 
  • Bronchodilator therapy may be indicated if proven obstruction
  • Long-term oxygen therapy in advanced disease (criteria pO2 < 7.3, or pO2 <7.8 with secondary polycythaemia, pulmonary hypertension or) – under specialist respiratory supervision/guidance
  • CFTR modulators (e.g. ivacaftor/Kaftrio) for patients with cystic fibrosis – under specialist respiratory supervision/guidance
  • Non-invasive ventilation for severe disease – under specialist respiratory supervision/guidance
  • Bronchoscopy for bronchoalveolar lavage can be considered in patients with recurrent exacerbations and no sputum production
  • Immunoglobulin replacement therapy for patients with functional antibody and immunoglobulin deficiencies 
Surgical /interventional radiological management
  • Lobectomy – wedge resection be indicated for bronchiectatic segment (particularly in poorly controlled cases with a high frequency of exacerbation or refractory haemoptysis, or foreign body/tumour resection)
  • Bronchial artery embolisation for massive haemoptysis
  • Lung transplantation 

References

T Hill A, L Sullivan A, D Chalmers J, et al British Thoracic Society Guideline for bronchiectasis in adults Thorax 2019;74:1-69.

Written by Dr Anahita Sharma (IMT3)
Reviewed by Dr. Ashraf Elgaali (ST7 Respiratory Medicine)

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