What is the role of autoantibody tests in the workup of vasculitis?

Updated: Dec 10, 2018
  • Author: Nadia Jennifer Chiara Luca, MD; Chief Editor: Lawrence K Jung, MD  more...
  • Print

The initial test for antineutrophil cytoplasmic antibody (ANCA) is indirect immunofluorescence to detect staining pattern, either cytoplasmic or perinuclear.

If positive results are noted, test for reactivity to proteinase 3 (PR3) and myeloperoxidase (MPO) by enzyme-linked immunoassay (ELISA)

ANCA positivity is noted in approximately 90% of pediatric patients with granulomatosis with polyangiitis (GPA) (formerly Wegener granulomatosis), 79% of who are cytoplasmic. [6]

PR3-ANCA and MPO-ANCA positivity have a high sensitivity and specificity for the diagnosis of GPA and microscopic polyangiitis (MPA), respectively.

The association of Churg-Strauss syndrome and ANCA positivity is approximately 40%.

Note that an atypical ANCA finding is nonspecific and may be seen in other inflammatory conditions such as infection and inflammatory bowel disease.

Anti-glomerular basement membrane (GBM) antibody testing is indicated for pulmonary renal syndromes.

Other autoantibodies such as anti-nuclear antibody (ANA) and rheumatoid factor (RF) are rarely positive

Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!