Clinical outcome after acquisition of Burkholderia cepacia in patients with cystic fibrosis.

Abstract

Respiratory disease is the major cause of morbidity and mortality in cystic fibrosis (CF). The significance of Burkholderia cepacia (B. cepacia) in the pathogenesis of lung disease in CF is debated, but its exact role remains unclear. To assess the impact of respiratory tract colonisation with B. cepacia in patients with CF by measuring changes in pulmonary function and body mass index (BMI). Three groups of patients were defined based on sputum culture isolates: Group 1 were B. cepacia and Pseudomonas aeruginosa (P. aeruginosa) positive patients; Group 2 were P. aeruginosa positive; and Group 3 were colonised with neither organism. Forced expiratory volume (FEV) and BMI were measured annually from 1987 to 1995 and the year of acquisition of P. aeruginosa or B. cepacia was recorded. The mean annual decrease in FEV1 was significantly different in all three groups: Group 1, -5.4 (5.1)%; Group 2, -3.9 (6.5)%; and Group 3, -1.6 (1.0)%, (p<0.05). The mean percentage decrease in FEV1 of a sub-group of Group 1 patients where the B. cepacia acquisition date was known was 6.1% per year versus 1.55% in Group 2 patients (p<0.05). Acquisition of B. cepacia may be a cause of, rather than a marker for, a decrease in pulmonary function.