Characterisation of the "Frequent Exacerbator Phenotype" in Bronchiectasis.

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Abstract

RATIONALE

Exacerbations are key events in the natural history of bronchiectasis, but clinical predictors and outcomes of frequently exacerbating patients are not well described.

OBJECTIVES

To establish if there is a "frequent exacerbator phenotype" in bronchiectasis and the impact of exacerbations on long-term clinical outcomes.

METHODS

We studied bronchiectasis patients enrolled from 10 clinical centres in Europe and Israel, with up to 5-years follow-up. Patients were categorized by baseline exacerbation frequency (0, 1, 2 or >3 per year). The repeatability of exacerbation status was assessed as well as the independent impact of exacerbation history on hospitalizations, quality of life and mortality.

MEASUREMENTS AND MAIN RESULTS

2572 patients were included. Frequent exacerbations were the strongest predictor of future exacerbation frequency suggesting a consistent "phenotype". The incident rate ratios for future exacerbations were 1.73 (95%CI 1.47-2.02, p<0.0001) for 1 exacerbation per year, 3.14 (95%CI 2.70-3.66, p<0.0001) for 2 exacerbations and 5.97 (95%CI 5.27-6.78, p<0.0001) for patients with >3 exacerbations per year at baseline. Additional independent predictors of future exacerbation frequency were Haemophilus influenzae and Pseudomonas aeruginosa infection, forced expiratory volume in 1 second, radiological severity of disease and co-existing COPD. Frequently exacerbating patients had worse quality of life and were more likely to be hospitalized during follow-up. Mortality over up to 5 years follow-up increased with increasing exacerbation frequency.

CONCLUSIONS

The frequent exacerbator phenotype in bronchiectasis is consistent over time and shows high disease severity, poor quality of life and increased mortality during follow-up.

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