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Pneumothorax in Cystic Fibrosis^*

^* From the Departments of Medicine (Drs. Flume and Strange) and Biometry and Epidemiology (Dr. Ye and Ms. Clark) and Pediatrics (Ms. Ebeling and Dr. Hulsey), Medical University of South Carolina, Charleston, SC.

Correspondence to: Patrick A. Flume, MD, FCCP, Medical University of South Carolina, 96 Jonathan Lucas St, 812-CSB, Charleston, SC 29425; e-mail: flumepa{at}musc.edu

Background: Spontaneous pneumothorax is a complication that is 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.

Objective: To identify risk factors associated with pneumothorax and to determine the prognosis of CF patients following an episode of pneumothorax.

Design: A retrospective observational cohort study of the National Cystic Fibrosis Patient Registry between the years 1990 and 1999.

Patients: The registry contained data on 28,858 patients with CF who had been followed up over those10 years at CF centers across the United States.

Results: Pneumothorax occurred with an average annual incidence of 0.64% and in 3.4% of patients overall. There was no increased occurrence by sex, but CF was more prevalent in older patients (mean [± SD] age, 21.9 ± 9.1 years) with more severe pulmonary impairment (nearly 75% of patients with FEV₁ of < 40% predicted). The principal risks associated with an increased occurrence of pneumothorax included the presence of Pseudomonas aeruginosa (odds ratio [OR], 2.3), Burkholderia cepacia (OR, 1.8), or Aspergillus (OR, 1.3) in sputum cultures, FEV₁ < 30% predicted (OR, 1.5), enteral feeding (OR, 1.7), Medicaid insurance (OR, 1.1), pancreatic insufficiency (OR, 1.4), allergic bronchopulmonary aspergillosis (OR, 1.5), and massive hemoptysis (OR, 1.4). There is an increased morbidity (eg, increased number of hospitalizations and number of days spent in the hospital) and an increased 2-year mortality rate following pneumothorax.

Conclusion: Pneumothorax is a serious complication in CF patients, occurring more commonly in older patients with more advanced lung disease. Nearly 1 in 167 patients will experience 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.

Key Words: cystic fibrosis • lung disease • pneumothorax

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