Massive hemoptysis in cystic fibrosis

Abstract

Background: Massive hemoptysis is a complication commonly reported in patients with cystic fibrosis (CF). An understanding of the pathophysiology of this complication and its consequences is important for the management of patients with CF.

Objectives: To identify risk factors associated with massive hemoptysis, and to determine the prognosis of patients following an episode of massive hemoptysis.

Design: A retrospective, observational cohort study of the National CF Patient Registry between the years 1990 to 1999.

Patients: The Registry contained data on 28,858 patients with CF observed over 10 years at CF centers across the United States.

Results: Massive hemoptysis occurred with an average annual incidence of 0.87% and in 4.1% of patients overall. There was no increased occurrence by sex, but it was more prevalent in older patients (mean age, 24.2 +/- 8.7 years [+/- SD]) with more severe pulmonary impairment (nearly 60% of patients who had an episode of massive hemoptysis had FEV1 < 40% predicted). The principal risks associated with an increased occurrence of massive hemoptysis included the presence of Staphylococcus aureus in sputum cultures (odds ratio [OR], 1.3) and diabetes (OR, 1.1). There was an increased morbidity (eg, increased hospitalizations and hospital days) and an increased 2-year mortality following massive hemoptysis.

Conclusion: Massive hemoptysis is a serious complication in CF patients, occurring more commonly in older patients with more advanced lung disease. Nearly 1 in 100 patients will have this complication each year. There is an attributable mortality to the complication and considerable morbidity, resulting in increased health-care utilization and a measurable decline in lung function.