Autoantibody response to BPI predict disease severity and outcome in cystic fibrosis

Citations of this article
Mendeley users who have this article in their library.


Background: Autoantibodies against bactericidal permeability increasing protein (BPI-ANCA) are frequently present in cystic fibrosis patients and have been reported to be associated to colonization with Pseudomonas (P) aeruginosa and lung damage. In the present study, we investigated BPI-ANCA as a prognostic marker and its relation to P. aeruginosa colonization and lung function. Methods: BPI-ANCA, measured by ELISA, was examined relative to lung function and microbiological findings. The prognostic value of BPI-ANCA was assessed in 46 adult patients followed for 1.2-8.9 years. The cross-sectional investigation was performed in 366 patients (age 0.5-55). Results: The presence of BPI-ANCA predicted poor prognosis. An adverse outcome occurred in 15/28 BPI-ANCA positive patients and in 2/18 BPI-ANCA negative patients (p = 0.01). This result remained valid when the patients were stratified according to lung function (p = 0.03). Findings of BPI-ANCA were correlated to P. aeruginosa colonization and lung damage. Development of BPI-ANCA occurred after colonization with P. aeruginosa. All colonized patients did not develop BPI-ANCA. The BPI-ANCA levels were fairly stable during the disease course, but decreased significantly following lung transplantation. Conclusion: BPI-ANCA responses follow colonization with P. aeruginosa and may be predictive for lung damage. © 2006 European Cystic Fibrosis Society.




Carlsson, M., Eriksson, L., Pressler, T., Kornfält, R., Mared, L., Meyer, P., … Segelmark, M. (2007). Autoantibody response to BPI predict disease severity and outcome in cystic fibrosis. Journal of Cystic Fibrosis, 6(3), 228–233.

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Save time finding and organizing research with Mendeley

Sign up for free