BPI-ANCA and long-term prognosis among 46 adult CF patients: a prospective 10-year follow-up study.
BACKGROUND
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.


OBJECTIVE
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.
Department of Respiratory Medicine and Allergology, Lund University and Skane University Hospital, 221 85 Lund, Sweden.
Ulrika
U
Lindberg
Malin
M
Carlsson
Claes-Göran
CG
Löfdahl
Mårten
M
Segelmark
D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology* / Q000378:metabolism; D023181:Antimicrobial Cationic Peptides / Q000276:immunology* / Q000378:metabolism; D015415:Biomarkers / Q000378:metabolism; D001798:Blood Proteins / Q000276:immunology* / Q000378:metabolism; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000276:immunology* / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D054884:Host-Pathogen Interactions / Q000276:immunology; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology / Q000378:metabolism; D008168:Lung / Q000276:immunology / Q000378:metabolism / Q000382:microbiology; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D011552:Pseudomonas Infections / Q000276:immunology / Q000378:metabolism; D011550:Pseudomonas aeruginosa / Q000276:immunology; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D015415:Biomarkers; D001798:Blood Proteins; D007070:Immunoglobulin A; C021623:bactericidal permeability increasing protein