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Clinical and Developmental Immunology
Volume 2012, Article ID 370107, 8 pages
Research Article

BPI-ANCA and Long-Term Prognosis among 46 Adult CF Patients: A Prospective 10-Year Follow-Up Study

1Department of Respiratory Medicine and Allergology, Lund University and Skane University Hospital, 221 85 Lund, Sweden
2Department of Nephrology and Department of Clinical Sciences, Lund University, 221 85 Lund, Sweden
3Department of Medicine and Health, Linkoping University, 590 50 linkoping, Sweden
4Department of Nephrology UHL, County Council of Ostergotland, 581 85 Linkoping, Sweden

Received 27 September 2012; Accepted 22 October 2012

Academic Editor: Dimitrios P. Bogdanos

Copyright © 2012 Ulrika Lindberg et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Introduction. Anti-neutrophil cytoplasmic antibodies specific for bactericidal/permeability-increasing protein (BPI-ANCA) are frequent in CF patients and mainly develop in response to infection with Pseudomonas aeruginosa. It is not known to what extent BPI-ANCA correlates to prognosis. Objectives. To evaluate the prognostic value of IgA-BPI-ANCA, measured at the beginning of the study, for transplantation-free survival. Methods. A cohort of 46 adult, nontransplanted CF patients was generated, 1995–1998, and characterized using Leeds criteria, lung function, and IgA-BPI-ANCA levels measured by ELISA. The cohort was followed until December 2009, using the combined endpoint of death or lung transplantation. Results. Lung function and IgA-BPI-ANCA, but not Leeds criteria, were significantly associated with adverse outcome. No patient with normal lung function at baseline reached endpoint. Within 10 years 8/11 with high BPI-ANCA reached an endpoint compared to 3/17 ANCA-negative patients. A similar result was seen within the Leeds I group where 7 out of 9 BPI-ANCA-positive patients reached endpoint, compared to none of the 5 patients without BPI-ANCA. Conclusions. IgA-BPI-ANCA is associated with adverse outcome among Pseudomonas aeruginosa infected CF patients, suggesting that BPI-ANCA is a biomarker of an unfavourable host-pathogen interaction.