Cystic Fibrosis Carrier States Are Associated With More Severe Cases of Bronchiectasis

Abstract Background People with cystic fibrosis (CF) are at increased risk for bronchiectasis, and several reports suggest that CF carriers may also be at higher risk for developing bronchiectasis. The purpose of this study was to determine if CF carriers are at risk for more severe courses or complications of bronchiectasis. Methods Using MarketScan data (2001–2021), we built a cohort consisting of 105 CF carriers with bronchiectasis and 300 083 controls with bronchiectasis but without a CF carrier diagnosis. We evaluated if CF carriers were more likely to be hospitalized for bronchiectasis. In addition, we examined if CF carriers were more likely to be infected with Pseudomonas aeruginosa or nontuberculous mycobacteria (NTM) or to have filled more antibiotic prescriptions. We considered regression models for incident and rate outcomes that controlled for age, sex, smoking status, and comorbidities. Results The odds of hospitalization were almost 2.4 times higher (95% CI, 1.116–5.255) for CF carriers with bronchiectasis when compared with non–CF carriers with bronchiectasis. The estimated odds of being diagnosed with a Pseudomonas infection for CF carriers vs noncarriers was about 4.2 times higher (95% CI, 2.417–7.551) and 5.4 times higher (95% CI, 3.398–8.804) for being diagnosed with NTM. The rate of distinct antibiotic fill dates was estimated to be 2 times higher for carriers as compared with controls (95% CI, 1.735–2.333), and the rate ratio for the total number of days of antibiotics supplied was estimated as 2.8 (95% CI, 2.290–3.442). Conclusions CF carriers with bronchiectasis required more hospitalizations and more frequent administration of antibiotics as compared with noncarriers. Given that CF carriers were also more likely to be diagnosed with Pseudomonas and NTM infections, CF carriers with bronchiectasis may have a phenotype more resembling CF-related bronchiectasis than non-CF bronchiectasis. Using Marketscan data (2001-2021), we found that cystic fibrosis (CF) carriers with bronchiectasis face nearly 2.4 higher odds of hospitalization, increased rates of Pseudomonas and NTM infections and antibiotic use compared to non-CF-carriers with bronchiectasis suggesting a unique bronchiectasis phenotype.