ANCA against bactericidal/permeability-increasing protein, azurocidin, calprotectin and defensins in rheumatic and infectious diseases: prevalence and clinical associations

Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S117-20.


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.

Conclusion: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Antimicrobial Cationic Peptides
  • Biomarkers
  • Blood Proteins / immunology*
  • Carrier Proteins / immunology
  • Defensins / immunology*
  • Diagnosis, Differential
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Fluorescent Antibody Technique, Indirect
  • Humans
  • Infections / diagnosis
  • Infections / epidemiology
  • Infections / immunology*
  • Leukocyte L1 Antigen Complex / immunology*
  • Male
  • Membrane Proteins*
  • Middle Aged
  • Rheumatic Diseases / diagnosis
  • Rheumatic Diseases / epidemiology
  • Rheumatic Diseases / immunology*
  • Seroepidemiologic Studies


  • AZU1 protein, human
  • Antibodies, Antineutrophil Cytoplasmic
  • Antimicrobial Cationic Peptides
  • Biomarkers
  • Blood Proteins
  • Carrier Proteins
  • Defensins
  • Leukocyte L1 Antigen Complex
  • Membrane Proteins
  • bactericidal permeability increasing protein