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ANCA Screen with Reflex to ANCA Titer

ANCA Screen with Reflex to ANCA Titer

Test Highlight

ANCA Screen with Reflex to ANCA Titer


Clinical Use

  • Differential diagnosis of systemic necrotizing vasculitides

Clinical Background

Neutrophil cytoplasmic antibodies are associated with inflammatory diseases such as systemic necrotizing vasculitides and are therefore useful in the differential diagnosis. Specific tests for myeloperoxidase (MPO) and proteinase-3 (PR3) autoantibodies are recommended by an international consensus group.

Anti-MPO and anti-PR3 are useful in the classification of systemic vasculitides, including granulomatosis with polyangiitis (Wegener’s), microscopic polyarteritis (MPA), Churg-Strauss syndrome, syndromes of lung hemorrhage and nephritis, and primary pauci-immune necrotizing and crescentic glomerulonephritis. In addition, these autoantibodies are helpful in distinguishing active versus inactive disease states, monitoring the effect of therapy, and evaluating the possibility of relapse.

Anti-PR3 antibodies are found in 70% to 95% of patients with granulomatosis with polyangiitis (Wegener’s).

Anti-MPO antibodies are commonly associated with MPA, Churg-Strauss syndrome, and idiopathic crescentic glomerulonephritis (ICGN). Ninety percent of patients with inflammatory bowel disease, autoimmune hepatitis, or cholangitic liver disease are negative for MPO antibodies even though perinuclear staining of neutrophils may be observed. However, subtle differences in the pattern of staining of neutrophils fixed with ethanol versus formalin assist in the differentiation of MPO associated vasculitis (P-ANCA pattern) from inflammatory bowel disease (atypical P-ANCA pattern).

Close follow-up of MPO or PR3 antibody-positive patients is imperative because these patients may have, or be at risk for, serious progressive disease.


ANCA immunofluorescence assay; the immunofluorescence pattern and antibody titer are determined (at an additional charge, associated with an additional CPT code) for samples with positive results. This test does not specify the type of autoantibody present (eg, MPO or PR3 antibody). Separate tests codes are available for that purpose.

Interpretive Information

A positive ANCA screen with C-ANCA or P-ANCA pattern suggests a systemic necrotizing vasculitides. Correlation with clinical findings aids in diagnosis.


  1. Goekin JA, Bonsib SM. Anti-neutrophil cytoplasmic antibody and the histopathologic diagnosis of vasculitis. ASCP National Meeting, 1992.

  2. Hardarson S, Labrecque DR, Mitros FA, et al. Antineutrophil cytoplasmic antibody in inflammatory bowel and hepatobiliary diseases. High prevalence in ulcerative colitis, primary sclerosing cholangitis, and autoimmune hepatitis. Am J Clin Pathol. 1993;99:277-281.

  3. Jennette JC, Falk RJ. Antineutrophil cytoplasmic autoantibodies in inflammatory bowel disease. Am J Clin Pathol. 1993;99:221-223.

  4. Kallenberg CG, Mulder AH, Tervaert JW. Antineutrophil cytoplasmic antibodies: a still-growing class of autoantibodies in inflammatory disorders. Am J Med. 1992;93:675-682.

  5. Mandl LA, Solomon DH, Smith EL, et al. Using antineutrophil cytoplasmic antibody testing to diagnose vasculitis: can test-ordering guidelines improve diagnostic accuracy? Arch Intern Med. 2002;162:1509-1514.

  6. Savige J, Gillis D, Benson E, et al. International consensus statement on testing and reporting of antineutrophil cytoplasmic antibodies (ANCA). Am J Clin Pathol. 1999; 111:507-513.

Reflex tests are performed at an additional charge and are associated with an additional CPT code(s).

Content reviewed 04/2013

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