title: [Anti-Pseudomonas aeruginosa antibodies, circulating immune complexes, and anticytoplasm antibodies of neutrophils in patients with cystic fibrosis with and without Pseudomonas aeruginosa colonization].
abstract: Chronic lung infections, mainly due to Pseudomonas aeruginosa (Pa), account for the most of the morbidity and mortality in CF patients. The pathogenic factors predisposing to airway colonization are still nuclear. Host's immune response is not only poorly protective but can also act as a damaging factor in the development of the disease. Moreover, clinical manifestations of an overactive immune response, including vasculitis and arthropathy, have been recently described in the CF population. To deepen factors involved in the pathogenesis of lung injury, we evaluated the presence of anti-Pa precipitating antibodies, circulating immune complexes (CIC) and antineutrophil cytoplasmic antibodies (ANCA) in sera from 50 CF patients colonized and not by Pa. Number of anti-Pa precipitins was significantly different in chronically and in not colonized patients (p < 0.001; t = 7.75). Anti-Pa antibodies were positively correlated to age (p = 0.002, r = 0.42) and inversely correlated to lung function parameters (p = 0.031 r = -0.35 with respect to FVC). Mean C3-CIC levels in the sera were statistically higher in chronically colonized patients (p = 0.013; t = 2.57); while there was not a significant difference with respect to C1q-CIC values. Four patients, all chronically colonized by Pa, were ANCA-positive at indirect immunofluorescence, showing a cytoplasmic pattern. All 50 patients were found to be negative when tested for anti-mpo and anti-pr3 antibodies with ELISA. In conclusion, our data demonstrate that persistence of Pa provides a stimulus for chronic inflammation and the immune response in CF patients, leading to anti-Pa antibodies and CIC production. Even in the face of further research, we speculate that c-ANCA production is secondary to neutrophil activation with a consequent release of cytoplasmic enzymes that perpetuate and increase the inflammatory process. Since anti-mpo and anti-pr3 antibodies rarely occur in CF patients we suggest that other antigenic specificities may be involved in ANCA production.
authors.affiliation: Centro Fibrosi Cistica, Dipartimento di Pediatria, Università degli Studi di Firenze, Italia.
authors.forename: E
authors.initials: E
authors.lastname: Chiappini
authors.forename: G
authors.initials: G
authors.lastname: Taccetti
authors.forename: S
authors.initials: S
authors.lastname: Campana
authors.forename: S
authors.initials: S
authors.lastname: Turchini
authors.forename: L
authors.initials: L
authors.lastname: Marianelli
mesh_terms: D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D000907:Antibodies, Bacterial / Q000097:blood*; D000936:Antigen-Antibody Complex / Q000097:blood*; D002648:Child; D003550:Cystic Fibrosis / Q000097:blood* / Q000150:complications / Q000382:microbiology; D005260:Female; D006801:Humans; D008297:Male; D011552:Pseudomonas Infections / Q000097:blood* / Q000150:complications; D011550:Pseudomonas aeruginosa / Q000276:immunology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000907:Antibodies, Bacterial; D000936:Antigen-Antibody Complex