Table 1

Quick summary guide

Whom to investigate?Patients with persistent production of mucopurulent or purulent sputum particularly with relevant associated risk factors.
Patients with rheumatoid arthritis if they have symptoms of chronic productive cough or recurrent chest infections.
Patients with COPD with frequent exacerbations (two or more annually) and a previous positive sputum culture for P. aeruginosa whilst stable.
Patients with inflammatory bowel disease and chronic productive cough.
What radiology?Perform baseline chest X-ray.
Perform a thin section CT.
What tests?Co-morbidities and past medical history should be recorded.
Investigations for reflux and aspiration in symptomatic patients, or where there are other suggestive clinical features.
Measure Full blood count, serum total IgE and specific IgE or skin prick test to Aspergillus in all patients to investigate for Allergic Broncho Pulmonary Aspergillosis.
Measure serum IgG, IgA and IgM in all patients with bronchiectasis to exclude immunodeficiency.
Consider measuring baseline specific antibody levels against capsular polysaccharides of S. pneumoniae in all patients to investigate for specific antibody deficiency. If pneumococcal antibodies are low, immunise with 23 valent polysaccharide pneumococcal vaccine, followed by measurement of specific antibody levels 4-8 weeks later.
Test for cystic fibrosis (according to NICE Guidelines for CF) in patients with supporting clinical features.
Test for primary ciliary dyskinesia (PCD) in patients with supporting clinical features. Measurement of nasal nitric oxide is the first line investigation.
Send sputum cultures in all patients with bronchiectasis for routine and mycobacterial culture whilst clinically stable.
Airways clearanceManagement in stable disease- See figure 3
Management in Exacerbations- See figure 4
See figure 2
Antibiotic treatment for exacerbations- See table 6
Management of rhinosinusitis- See figure 5
Management of ABPA:
Offer oral corticosteroid to patients with active ABPA. An initial dose of 0.5 mg/kg/d for 2 weeks is recommended. Wean steroids according clinical response and serum IgE levels.
Consider itraconazole as a steroid sparing agent for patients dependent on oral corticosteroids where difficulty in weaning is experienced.
Monitor with total IgE level to assess treatment response.
The Deteriorating PatientA deteriorating patient is defined as significant and prolonged deterioration of symptoms, unexpected increased frequency or severity of exacerbations, frequent hospital admissions, early relapse after treatment of an exacerbation or rapid decline in lung function. A management plan to help in dealing with these patients is shown in figure 1.
Who should be followed up in secondary carePatients with chronic Pseudomonas aeruginosa, non-tuberculous mycobacteria (NTM) or methicillin-resistant Staphylococcus aureus colonisation (MRSA);
Deteriorating bronchiectasis with declining lung function;
Recurrent exacerbations (≥3 per year);
Patients receiving long term antibiotic therapy (oral, inhaled or nebulised);
Patients with bronchiectasis and associated rheumatoid arthritis, immune deficiency, inflammatory bowel disease and primary ciliary dyskinesia;
Patients with allergic bronchopulmonary aspergillosis;
Patients with advanced disease and those considering transplantation.
MonitoringSee table 7
See tables 4 and 5 for severity assessment