Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis.
BACKGROUND
Bactericidal-permeability-increasing protein (BPI) is a potent anti-microbial protein produced by neutrophil granulocytes. Anti-neutrophil cytoplasmatic antibodies (ANCA) directed against BPI have been detected in up to 91% in patients with cystic fibrosis (CF). We aimed to evaluate the prevalence of BPI-ANCA in our CF patients and to determine whether presence of BPI-ANCA is correlated with organ damage.


METHODS
Twenty-four patients performed respiratory function testing and pulmonary high-resolution computed tomography (HRCT). HRCT was scored by using a modified Bhalla method. Serum samples were analysed by direct binding enzyme-linked immunosorbent assay for BPI-ANCA.


RESULTS
The prevalence of anti-BPI-IgG was 71% and anti-BPI-IgA 33%. Twenty-nine percent of our patients were positive for both BPI-ANCA isotypes. Mean HRCT score was 8.0 ranging from 0 to 22, bronchiectasis presented the most common finding (79%). There was a significant correlation between BPI-ANCA and both HRCT score and FEV(1) (p < 0.01). High levels of BPI-ANCA were correlated to chronic Pseudomonas aeruginosa lung infection (p < 0.01).


CONCLUSIONS
BPI-ANCA was common in our study group. Highly significant correlations between BPI-ANCA and parameters to evaluate lung disease in CF may be a consequence of the inflammation process, or it may indicate a pathogenic role of BPI-ANCA levels in the development of lung disease. More research is needed and the clinical significance of our findings needs further evaluation.
Dorlöchter
Ludger
L
Department of Paediatrics, Haukeland University Hospital, N-5021 Bergen, Norway. ludger.dorlochter@helse-bergen.no
Carlsson
Malin
M
Olafsdottir
Edda J
EJ
Røksund
Ola D
OD
Rosendahl
Karen
K
Fluge
Gjermund
G
D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology*; D023181:Antimicrobial Cationic Peptides; D001798:Blood Proteins / Q000276:immunology*; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000276:immunology* / Q000503:physiopathology*; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D014057:Tomography, X-Ray Computed / Q000379:methods
D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D001798:Blood Proteins; D008565:Membrane Proteins; C021623:bactericidal permeability increasing protein