title: ANCA against bactericidal/permeability-increasing protein, azurocidin, calprotectin and defensins in rheumatic and infectious diseases: prevalence and clinical associations.
abstract: OBJECTIVE
  To determine the prevalence and clinical associations of ANCA against the antibiotic proteins and peptides: Bactericidal/permeability-increasing protein (BPI), Azurocidin (AZ), Calprotectin (CP) and beta-Defensin-1 and -2 (DF).
  
  
  METHODS
  Patients with ANCA-associated vasculitides (n = 99), other vasculitides and rheumatic connective tissue diseases (n = 303), HIV-infection (n = 66), other infectious diseases (n = 134) Crohn's disease (n = 12) and ulcerative colitis (n = 12) were tested for BPI-, AZ-, CP-, DF-, PR3-, and MPO-ANCA in indirect immunofluorescence technique (IFT) and ELISA.
  
  
  RESULTS
  In ANCA associated vasculitides BPI-ANCA were detected in 6% of patients. In HIV infection, BPI was the main target antigen of ANCA-IFT positive sera (74%). BPI-ANCA was associated with higher inflammatory activity. In Crohn's disease and ulcerative colitis BPI-ANCA was prominent (34% of patients). AZ-ANCA were found in 5% of patients. No ANCA were detected against defensin and calprotectin.
  
  
  CONCLUSIONS
  BPI-ANCA is the main autoantibody in HIV and is associated with higher inflammatory activity. In inflammatory bowel diseases BPI-ANCA is predominant, AZ-ANCA are also present to a lesser extend. Both were not useful characterize clinical subgroups. No ANCA were detected against calprotectin or defensins.
authors.affiliation: Department of Rheumatology, University of Lübeck, Lübeck, Germany.
authors.forename: H
authors.initials: H
authors.lastname: Schultz
authors.forename: E
authors.initials: E
authors.lastname: Csernok
authors.forename: K
authors.initials: K
authors.lastname: Herlyn
authors.forename: P H
authors.initials: PH
authors.lastname: Reichel
authors.forename: F
authors.initials: F
authors.lastname: Moosig
authors.forename: O A
authors.initials: OA
authors.lastname: Cornely
authors.forename: M K
authors.initials: MK
authors.lastname: Fagerhol
authors.forename: W L
authors.initials: WL
authors.lastname: Gross
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D023181:Antimicrobial Cationic Peptides; D015415:Biomarkers; D001798:Blood Proteins / Q000276:immunology*; D002352:Carrier Proteins / Q000276:immunology; D023082:Defensins / Q000276:immunology*; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D007239:Infections / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*; D039841:Leukocyte L1 Antigen Complex / Q000276:immunology*; D008297:Male; D008565:Membrane Proteins; D008875:Middle Aged; D012216:Rheumatic Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology*; D016036:Seroepidemiologic Studies
chemical_list: C062991:AZU1 protein, human; D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D015415:Biomarkers; D001798:Blood Proteins; D002352:Carrier Proteins; D023082:Defensins; D039841:Leukocyte L1 Antigen Complex; D008565:Membrane Proteins; C021623:bactericidal permeability increasing protein