Anti-neutrophil cytoplasmic antibodies directed against the bactericidal/permeability-increasing protein (BPI) in pediatric cystic fibrosis patients do not recognize N-terminal regions important for the anti-microbial and lipopolysaccharide-binding activity of BPI

摘要: This study was performed to examine the prevalence and clinical correlates of bactericidal/permeability-increasing protein anti-neutrophil cytoplasmic antibodies (BPI-ANCA) in pediatric cystic fibrosis (CF) patients elucidate their possible role CF pulmonary pathology. Sera 27 were tested for ANCA by indirect immunofluorescence (IFT) enzyme-linked immunosorbent assay (ELISA) sub-specificities. BPI-ANCA examined using standard ELISA BPI, lipopolysaccharide-binding (LBP), BPI/LBP fusion proteins epitope map main binding sites look cross-reactivity with LBP. Pulmonary function serum concentrations total immunoglobulin G (IgG) measured infections diagnosed. In addition, release reactive oxygen species (ROS) neutrophil granulocytes after stimulation monoclonal BPI-ANCA. Using IFT, two showed atypical staining, six exhibited perinuclear no staining detected. Of patients, 13 (48%) positive, three also A (IgA) positive; one patient had against lactoferrin; proteinase 3 detected any patients. All bound C-terminal region molecule; none N-terminus or There significant correlation between data occurrence this cross-sectional study. Release ROS from induced Activation neutrophils modulation BPI-mediated opsonophagocytosis disposal Gram-negative bacteria lipopolysaccharides raise possibility that they contribute pathology but intervention longitudinal studies large groups are needed establish a causative association.