Prevalence of antineutrophil cytoplasmic antibodies in patients with various pulmonary diseases or multiorgan dysfunction

Arthritis Rheum. 2003 Apr 15;49(2):151-5. doi: 10.1002/art.10997.


Objective: To determine the prevalence of antineutrophil cytoplasmic antibodies (ANCA) in patients with diseases that may mimic systemic vasculitides, such as severe multiorgan dysfunction (MOD) and parenchymal pulmonary disorders.

Methods: We conducted a prospective study of patients with MOD admitted to the medical intensive care unit and patients with various lung diseases seen at the outpatient pulmonary clinic of a tertiary care hospital. Patients with a documented diagnosis of Wegener's granulomatosis (WG) served as positive controls. ANCA were determined in serum samples from each patient by a combination of indirect immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) for antibodies to proteinase-3 and myeloperoxidase (anti-MPO).

Results: Ninety-nine patients with MOD, 29 outpatients with various lung disorders, and 18 patients with WG were included in the study. ANCA were detected by IIF alone in 16% (15/96) of patients with nonvasculitic MOD and 17% (5/29) of outpatients with various pulmonary disorders. The majority of the positive IIF specimens from each group displayed an atypical IIF pattern (73% and 80%, respectively). Only 1 specimen from patients with nonvasculitic disorders was positive for anti-MPO. ANCA by both IIF and ELISA were detected in 78% (12/14) of control patients with WG.

Conclusion: Detection of ANCA by the combination of IIF and antigen-specific assays for proteinase 3 and myeloperoxidase in diseases that mimic systemic vasculitides is highly specific for WG, microscopic polyangiitis, and Churg-Strauss syndrome.

Publication types

  • Clinical Trial

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Antibody Specificity
  • Critical Care
  • Enzyme-Linked Immunosorbent Assay
  • False Positive Reactions
  • Fluorescent Antibody Technique, Indirect
  • Granulomatosis with Polyangiitis / epidemiology
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Inpatients
  • Lung Diseases / epidemiology*
  • Lung Diseases / immunology*
  • Multiple Organ Failure / epidemiology*
  • Multiple Organ Failure / immunology*
  • Outpatients
  • Prospective Studies
  • Seroepidemiologic Studies


  • Antibodies, Antineutrophil Cytoplasmic