Audience

  • NHS Highland
  • Primary and Secondary Care
  • Adults only

Bronchiectasis is a chronic respiratory condition presenting with frequent cough, chronic sputum production and multiple exacerbations per year. The key parenchymal structural abnormality is dilated bronchi often affecting multiple lobes. Bronchiectasis shares some clinical features with obstructive airways disease and patients often require multiple visits to primary and secondary care teams.

It is estimated that over 300,000 people in the UK are living with bronchiectasis with an overall 20% increase in prevalence over the last 10 years. Bronchiectasis is more common in females with an 8-to-10-fold increase in prevalence in adults over the age of 60 (300 to 500 cases per 100,000) when compared with those under 50 years of age (40 to 50 cases per 100,000). Bronchiectasis is thought to account for 1.4% of all deaths associated with lung disease worldwide. Patients with bronchiectasis require input from extensive healthcare resources, with bronchiectasis associated health care usage increasing with frequent antibiotic requirement, hospitalisation and primary care visits.

Pathophysiology

The induction and proliferation of bronchiectasis requires consideration of four impacting factors:

  1. Airways dysfunction
  2. Inflammatory response
  3. Structural disease
  4. Infection


The components of these factors form a complex interface that is described in the literature as a “vicious vortex” in contrast with a cyclical process that can be interrupted with a single intervention. Impaired mucociliary clearance and retention of airway secretion is thought to disrupt normal host defences, which render the airways susceptible to infection, often becoming persistent. This process, in turn, incites an inflammatory response that ultimately leads to bronchiectasis though airway remodelling.

Known causative aetiologies include:

  • Airway obstruction (e.g. foreign body aspiration)
  • Defective host defences
  • Cystic fibrosis
  • Young syndrome
  • Rheumatic and autoimmune diseases
  • Primary ciliary dyskinesia
  • Pulmonary infections
  • Allergic bronchopulmonary aspergillosis.

A report from the European Bronchiectasis Network (EMBARC) listed the following aetiologies as most commonly contributing to the development of bronchiectasis:

  • Post respiratory infection (60%)
  • Childhood infections (21%)
  • Chronic Obstructive Pulmonary Disease (8.1%)
  • Mycobacterial disease (5.9%)
  • Rheumatic disorders (4.8%)
  • Immunodeficiency (4.1%)

Clinical manifestation

Clinical features associated with bronchiectasis are: 

  • Cough most days of the week (98% of patients)
  • Production of mucopurulent sputum most days of the week for months to years (78% of patients)
  • AND a history of exacerbations.

Other, less specific symptoms include: dyspnoea, wheezing and pleuritic chest pain.

On examination, 75% of patients will have crepitations, 22% wheeze and only 2% demonstrating digital clubbing[4].

Assessment and diagnosis

Assessment

addition to thorough clinical examination and history taking:

  • Baseline chest x-ray
  • Blood tests (including full blood count, urea and electrolytes, C-reactive protein and liver function tests)
  • Sputum culture (for microscopy, culture, sensitivity and Alcohol and Acid Fast Bacillus AAFB) 

Referral

To be made to general respiratory clinic for full evaluation.

Diagnosis

Diagnosis of bronchiectasis is made clinically in the general respiratory clinic with presence of the following features:

  • cough on most days of the week
  • sputum production on most days of the week
  • history of exacerbations

Differential diagnosis

Where there is uncertainty, or other respiratory conditions are required to be ruled out, cross sectional imaging may be requested by a respiratory specialist.

Radiologic features on high resolution CT scan that are suggestive of bronchiectasis are:

  • Airway to arterial ratio ≥1.5 (internal airway lumen diameter/adjacent pulmonary artery diameter)
  • Lack of tapering of bronchi (tram track appearance)
  • Airway visibility within 1cm of a costal pleural surface or touching the mediastinal pleura

Post diagnosis testing

Once a diagnosis of bronchiectasis is made, efforts should be directed at determining the underlying cause. The general respiratory clinic will carry out testing for rheumatoid factor, anti-cyclic citrullinated peptide (Anti-CCP), antinuclear antibodies (ANA) and anti-neutrophil cytoplasmic antibodies (ANCA). Where patients have undergone radiologic imaging, a Bronchiectasis Severity Index (BSI) calculation will be carried out to guide clinical discussions: Bronchiectasis Severity Index - Bronchiectasis.

A sub-group of patients with complex disease will require review in the multi-disciplinary complex bronchiectasis clinic, this clinic is for those who have 2 of:

  • 3 or more microbiologically proven exacerbations with MRSA or Pseudomonas aeruginosa.
  • Intractably symptomatic bronchiectasis, despite optimal primary care management or general respiratory review
  • Structural bronchiectasis on cross sectional imaging

This clinic will establish a chronic management plan, advise on managing acute exacerbations and revisit chest clearance techniques. Patients who have any 2 of the above, and a pre-existing diagnosis of bronchiectasis, may be referred directly to the complex service.

Prognosis

Mild bronchiectasis (BSI 0 to 4):
1 year outcomes: mortality rate: 0 to 2.8%, hospitalisation rate: 0 to 3.4% 
4 year outcomes: mortality rate: 0 to 5.3%, hospitalisation rate: 0 to 9.2% 

Moderate bronchiectasis (BSI 5 to 8):
1 year outcomes: mortality rate 0.8 to 4.8 %, hospitalisation rate: 1.0 to 7.2% 
4 year outcomes: mortality rate: 4 to 11.3 %, hospitalisation rate: 9.9 to 19.4% 

Severe bronchiectasis (BSI >9):
1 year outcomes: mortality rate: 7.6 to 10.5%, hospitalisation rate: 16.7 to 52.6% 
4 year outcomes: mortality rate: 9.9 to 29.2%, hospitalisation rate: 41.2 to 80.4% 

Management in Primary Care

  1. Sputum sample (Microscopy, Culture and Sensitivity and AAFB if first presentation) if experiencing symptoms of exacerbation.
  2. Review airway clearance and consider referral to Physiotherapy
  3. Medications review (eg, clarification on any drug allergies, review of inhaler technique, previously used antibiotics)
  4. Referral to online resource for patient information: Respiratory resource hub
  5. Ensure up to date immunisations, including pneumococcal

For NHS Highland antibiotic guidance: Bronchiectasis (Antimicrobial)

Referral

Referral to general respiratory clinic if:

  • New diagnosis bronchiectasis suspected
  • Respiratory symptoms which require secondary care assessment

Referral to complex bronchiectasis service is indicated if:

  • 3 or more microbiologically proven exacerbations, lasting several days or who may necessitate intravenous or nebulised therapy
  • Bronchiectasis secondary to immunodeficiency or Primary Ciliary Dyskinesia
  • Those on nebulised antibiotic therapy
  • Those with Methicillin Resistant Staphylococcus aureus (MRSA) or Pseudomonas aeruginosa cultured in sputum
  • Concern regarding potential diagnosis of cystic fibrosis (eg, male infertility, family history, malabsorption, pancreatitis)

Complex bronchiectasis clinic is NOT for those who:

  • Have chronic obstructive pulmonary disease with superimposed bronchiectasis (unless phenotype meets one of the above criteria)
  • Require confirmation of diagnosis
  • Grow non-pathogenic organisms such as Pseudomonas putida or Pseudomonas fluoroscens

Referral process: 

  • Refer to Respiratory via SCI Gateway
  • All cases will be discussed at monthly MDT and triaged appropriately.

Management in Secondary Care

  • Triage and review process, IV antibiotic pathway and pathway for stopping nebulised antibiotics. See 
    NHS Highland Complex Bronchiectasis Protocols.
  • Immunoglobulin levels, specific antigens to Haemophilus influenzae and pneumococcus will be carried out on assessment in secondary care.

Specialist Respiratory Consultant advice should be sought, if a patient diagnosed with bronchiectasis has any of the following features:

  • 3 or more exacerbations, lasting several days or with complex organisms (ie, Pseudomonas aeruginosa), which may necessitate IV or nebulised therapy.
  • Bronchiectasis secondary to immunodeficiency or Primary Ciliary Dyskinesia
  • On nebulised antibiotic therapy
  • Intractable symptoms despite optimal management in primary care
  • Methicillin Resistant Staphylococcus aureus (MRSA) in sputum
  • Concern regarding potential diagnosis of cystic fibrosis (eg, male infertility, family history, malabsorption, pancreatitis)

Further information for Health Care Professionals

Patient and self-management information

Patient information

Self-management information

Editorial Information

Last reviewed: 27/06/2024

Next review date: 30/06/2027

Author(s): Respiratory.

Version: 1

Approved By: TAMSG of the ADTC

Reviewer name(s): L Allan, Physician Associate in Respiratory Medicine, Dr G Christie, Consultant Respiratory Physician, L Blaikie, Cystic Fibrosis Clinical Nurse Specialist.

Document Id: TAM633