Hemoptysis and serious outcomes in cystic fibrosis

Hemoptysis is a common and important complication in persons with cystic fibrosis (PwCF). Despite this, there is limited literature on the impact of hemoptysis on contemporary cystic fibrosis (CF) outcomes. We sought to evaluate whether hemoptysis increases risk of lung transplant or death without transplant in PwCF. We reviewed a dataset of PwCF ages 12 years or older from the CF Foundation Patient Registry (CFFPR) that included 29,587 individuals. We identified hemoptysis as our predictor of interest and categorized PwCF as either no hemoptysis, any hemoptysis (submassive and/or massive), or massive hemoptysis. We subsequently evaluated whether hemoptysis, as defined above, was associated with death without transplant or receipt of lung transplant via relative risk regression. We adjusted for age, sex, body mass index, forced expiratory volume in one second (FEV1), number of exacerbations, supplemental oxygen use, CF-related diabetes, and Pseudomonas aeruginosa colonization status. Subgroup analyses were performed in advanced lung disease, defined as PwCF with an FEV1 < 40% of predicted. We found PwCF with any form of hemoptysis were more likely to progress to lung transplant or die without transplant than PwCF who did not have hemoptysis [RR = 1.16 (95% CI 1.03, 1.30)]. The effect size of these associations was larger for hemoptysis events classified as “massive” [massive hemoptysis OR = 1.30 (95% CI 1.01, 1.68)], or in PwCF with advanced lung disease [massive hemoptysis in advanced lung disease OR = 1.29 (95% CI 1.03, 1.60)]. In conclusion, hemoptysis is associated with increased risk of lung transplant and death without transplant in PwCF, especially among those with massive hemoptysis or advanced lung disease. Our results suggest that hemoptysis functions as a useful predictor of serious outcomes in PwCF and may be important to incorporate into risk prediction models and/or transplant decisions in CF.