Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis

• Published in: Journal of cystic fibrosis Vol. 3; no. 3; pp. 179 - 183
• Main Authors: Dorlöchter, Ludger, Carlsson, Malin, Olafsdottir, Edda J., Røksund, Ola D., Rosendahl, Karen, Fluge, Gjermund
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier B.V 01.08.2004
• Subjects: Adolescent; Adult; Antibodies, Antineutrophil Cytoplasmic - blood; Antibodies, Antineutrophil Cytoplasmic - immunology; Antimicrobial Cationic Peptides; Autoantibodies; Blood Proteins - immunology; Child; Cystic Fibrosis - complications; Cystic Fibrosis - immunology; Cystic Fibrosis - physiopathology; Disease progression; Female; Forced Expiratory Volume; Humans; Lung Diseases - diagnosis; Lung Diseases - etiology; Lung Diseases - immunology; Lung Diseases - physiopathology; Male; Membrane Proteins - immunology; Spirometry; Tomography, X-Ray Computed - methods; X-ray tomography; X-ray tomography; Spirometry; Autoantibodies; Disease progression
• ISSN: 1569-1993; 1873-5010
• DOI: 10.1016/j.jcf.2004.04.005

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.