title: Prevalence and clinical significance of auto-antibodies in adults with cystic fibrosis.
abstract: The aim of this study was to determine the prevalence of different auto-antibodies in adult, French cystic fibrosis (CF) patients and to look for a correlation between autoimmunity, patient characteristics and survival. The sera of 144 patients were screened for a wide range of antibodies. Clinical, biological and bacteriological characteristics and the cystic fibrosis transmembrane conductance regulator genotype were recorded and progression of lung disease was examined. 113 (78.5%) patients displayed one or several auto-antibodies, predominantly immunoglobulin (Ig)A anti-Saccharomyces cerevisiae antibodies (ASCA; 43.7%) and antineutrophil cytoplasmic antibodies (ANCA; 40%), of which 59% showed bactericidal/permeability-increasing protein (BPI) specificity. The presence of BPI-ANCA was associated with the number of antibiotic courses, low body mass index, Pseudomonas aeruginosa colonisation, the presence of resistant P. aeruginosa, low forced expiratory volume in 1 s, CF-related liver disease, hypergammaglobulinaemia, male sex and inflammatory syndrome. The presence of ASCA-IgA was correlated with male sex and hypergammaglobulinaemia. 41 patients presented with chronic respiratory failure and/or requested lung transplantation or died during follow-up. These events were more frequent in patients with BPI-ANCA or ASCA-IgA. These findings confirm the high frequency of auto-antibodies in CF, particularly BPI-ANCA and ASCA-IgA, and the link between BPI-ANCA, severity of lung disease and CF prognosis.
authors.affiliation: Dept of Internal Medicine, Centre Hospitalier Lyon Sud, F-69495 Pierre-Bénite Cedex, France. lachenal@lyon.fnclcc.fr
authors.forename: F
authors.initials: F
authors.lastname: Lachenal
authors.forename: K
authors.initials: K
authors.lastname: Nkana
authors.forename: R
authors.initials: R
authors.lastname: Nove-Josserand
authors.forename: N
authors.initials: N
authors.lastname: Fabien
authors.forename: I
authors.initials: I
authors.lastname: Durieu
mesh_terms: D000293:Adolescent; D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000737:chemistry; D001323:Autoantibodies / Q000737:chemistry*; D015331:Cohort Studies; D003550:Cystic Fibrosis / Q000097:blood / Q000453:epidemiology / Q000235:genetics / Q000276:immunology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005838:Genotype; D006801:Humans; D007070:Immunoglobulin A / Q000737:chemistry; D008297:Male; D015995:Prevalence; D012189:Retrospective Studies; D012441:Saccharomyces cerevisiae / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D007070:Immunoglobulin A; D019005:Cystic Fibrosis Transmembrane Conductance Regulator