title: Autoantibody response to BPI predict disease severity and outcome in cystic fibrosis.
abstract: 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.
  
  
  CONCLUSIONS
  BPI-ANCA responses follow colonization with P. aeruginosa and may be predictive for lung damage.
authors.affiliation: Department of Laboratory Medicine, Section of Microbiology, Immunology and Glycobiology, Lund University, S-221 85 Lund, Sweden. malin.carlsson@med.lu.se
authors.forename: Malin
authors.initials: M
authors.lastname: Carlsson
authors.forename: Leif
authors.initials: L
authors.lastname: Eriksson
authors.forename: Tania
authors.initials: T
authors.lastname: Pressler
authors.forename: Ragnhild
authors.initials: R
authors.lastname: Kornfält
authors.forename: Lena
authors.initials: L
authors.lastname: Mared
authors.forename: Peter
authors.initials: P
authors.lastname: Meyer
authors.forename: Allan
authors.initials: A
authors.lastname: Wiik
authors.forename: Jörgen
authors.initials: J
authors.lastname: Wieslander
authors.forename: Mårten
authors.initials: M
authors.lastname: Segelmark
mesh_terms: D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D023181:Antimicrobial Cationic Peptides / Q000276:immunology*; D015415:Biomarkers; D001798:Blood Proteins / Q000276:immunology*; D002353:Carrier State / Q000276:immunology / Q000382:microbiology*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000276:immunology / Q000382:microbiology*; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D007223:Infant; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D008875:Middle Aged; D011379:Prognosis; D011550:Pseudomonas aeruginosa / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D015415:Biomarkers; D001798:Blood Proteins; D008565:Membrane Proteins; C021623:bactericidal permeability increasing protein