BPI-ANCA Provides Additional Clinical Information to Anti-Pseudomonas Serology: Results from a Cohort of 117 Swedish Cystic Fibrosis Patients.
Patients with cystic fibrosis (CF) colonized with Pseudomonas aeruginosa (P. aeruginosa) have worse prognosis compared with patients who are not. BPI-ANCA is an anti-neutrophil cytoplasmic antibody against BPI (bactericidal/permeability increasing protein) correlating with P. aeruginosa colonization and adverse long time prognosis. Whether it provides additional information as compared to standard anti-P. aeruginosa serology tests is not known. 117 nontransplanted CF patients at the CF centre in Lund, Sweden, were followed prospectively for ten years. Bacterial colonisation was classified according to the Leeds criteria. IgA BPI-ANCA was compared with assays for antibodies against alkaline protease (AP), Elastase (ELA), and Exotoxin A (ExoA). Lung function and patient outcome, alive, lung transplanted, or dead, were registered. BPI-ANCA showed the highest correlation with lung function impairment with an r-value of 0.44. Forty-eight of the 117 patients were chronically colonized with P. aeruginosa. Twenty of these patients experienced an adverse outcome. Receiver operator curve (ROC) analysis revealed that this could be predicted by BPI-ANCA (AUC = 0.77), (p = 0.002) to a better degree compared with serology tests. BPI-ANCA correlates better with lung function impairment and long time prognosis than anti-P. aeruginosa serology and has similar ability to identify patients with chronic P. aeruginosa.
Department of Clinical Sciences in Lund, Section of Respiratory Medicine and Allergology, Lund University and Skane University Hospital, 221 85 Lund, Sweden.
Ulrika
U
Lindberg
Department of Clinical Sciences in Lund, Section of Nephrology, Lund University, 221 85 Lund, Sweden.
Malin
M
Carlsson
Department of Clinical Sciences in Lund, Section of Nephrology, Lund University, 221 85 Lund, Sweden.
Thomas
T
Hellmark
Department of Medicine and Health, Linköping University, 581 83 Linköping, Sweden ; Department of Nephrology UHL, County Council of Östergötland, 581 83 Linköping, Sweden.
Mårten
M
Segelmark
D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D023181:Antimicrobial Cationic Peptides / Q000276:immunology*; D001323:Autoantibodies / Q000276:immunology; D001798:Blood Proteins / Q000276:immunology*; D002648:Child; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000150:complications* / Q000235:genetics / Q000401:mortality / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D006579:Heterozygote; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D008297:Male; D009154:Mutation; D063868:Patient Outcome Assessment; D011552:Pseudomonas Infections / Q000209:etiology* / Q000401:mortality / Q000503:physiopathology; D012372:ROC Curve; D012129:Respiratory Function Tests; D013548:Sweden; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D001323:Autoantibodies; D001798:Blood Proteins; D007070:Immunoglobulin A; C021623:bactericidal permeability increasing protein; D019005:Cystic Fibrosis Transmembrane Conductance Regulator