Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis.

Autor: Dorlöchter L; Department of Paediatrics, Haukeland University Hospital, N-5021 Bergen, Norway. ludger.dorlochter@helse-bergen.no, Carlsson M, Olafsdottir EJ, Røksund OD, Rosendahl K, Fluge G
Jazyk: angličtina
Zdroj: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2004 Aug; Vol. 3 (3), pp. 179-83.
DOI: 10.1016/j.jcf.2004.04.005
Abstrakt: 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.
Databáze: MEDLINE
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