The development of a bronchiectasis severity score has been addressed in several studies with the purpose of modelling prognosis. The FACED score was designed to predict five-year mortality using five key variables:
• F: FEV1, score 0 to 2
• A: Age, score 0 to 2
• C: Colonisation with P. aeruginosa, score 0 to 1
• E: Extent of radiological involvement (number of lobes affected), score 0 to 1
• D: Dyspnoea (Medical Research Council dyspnoea scale), score 0 to 1.24
Three severity groups, mild (0 to 2 points), moderate (3 to 4 points) and severe (5 to 7 points), predicted five-year respiratory mortality of 0.9 to 2.4%, 13.6 to 15.3% and 45.6 to 51.6%, respectively. This is equivalent to five-year respiratory mortality rates of about one in 50 patients with mild disease, one in six patients with moderate disease and one in two patients with severe disease.
In this model, neither exacerbations (hospital admissions) nor nontuberculous mycobacterial colonisation were predictive of mortality, and age and FEV1 were the strongest mortality predictors. The FACED score and the similar, although slightly more detailed, Bronchiectasis Severity Index are being further evaluated for their ability to predict clinically important disease-related outcomes, including exacerbation frequency, quality of life, respiratory symptoms, functional capacity and lung function decline in bronchiectasis.25,26
Some patients with bronchiectasis will benefit from ongoing management by a respiratory physician. Specialist units may develop management plans at the time of diagnosis for both maintenance care and exacerbations. These plans help identify clear treatment goals and measures. Patient groups and conditions for which ongoing specialist management should be considered include:
• all children with bronchiectasis
• chronic P. aeruginosa, opportunist mycobacteria or methicillin-resistant S. aureus colonisation
• poor and declining lung function
• recurrent exacerbations (more than three per year)
• patients receiving or in need of prophylactic antibiotic therapy (oral or nebulised)
• complex associated comorbidity such as allergic bronchopulmonary aspergillosis, rheumatoid arthritis, immune deficiency, inflammatory bowel disease and primary ciliary dyskinesia
• patients in whom lung transplantation is being considered.
The objectives of good management in bronchiectasis are to:
• improve wellbeing
• reduce symptoms and exacerbations
• improve quality of life
• maintain or defer deterioration in lung function
• prolong survival.