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Test ID: ANCA Cytoplasmic Neutrophil Antibodies, Serum

Reporting Name

Cytoplasmic Neutrophilic Ab, S

Useful For

Antineutrophil cytoplasmic antibodies (cANCA and pANCA):

-Evaluating patients suspected of having autoimmune vasculitis (both Wegener granulomatosis [WG] and microscopic polyangiitis)

 

cANCA titer:

-May be useful for monitoring treatment response in patients with WG (systemic or organ-limited disease); increasing titer suggests relapse of disease, while a decreasing titer suggests successful treatment

Clinical Information

Antineutrophil cytoplasmic antibodies (ANCA) can occur in patients with autoimmune vasculitis including Wegener granulomatosis (WG), microscopic polyangiitis (MPA), or organ-limited variants thereof such as pauci-immune necrotizing glomerulonephritis.(2) Detection of ANCA is a well-established diagnostic test for the evaluation of patients suspected of having autoimmune vasculitis. ANCA react with enzymes in the cytoplasmic granules of human neutrophils including proteinase 3 (PR3), myeloperoxidase (MPO), elastase, and cathepsin G. Antibodies to PR3 occur in patients with WG (both classical WG and WG with limited end-organ involvement) and produce a characteristic pattern of granular cytoplasmic fluorescence on ethanol-fixed neutrophils called the cANCA pattern. Antibodies to MPO occur predominately in patients with MPA and produce a pattern of perinuclear cytoplasmic fluorescence on ethanol-fixed neutrophils called the pANCA pattern.

Interpretation

Positive results for antineutrophil cytoplasmic antibodies (cANCA or pANCA) are consistent with the diagnosis of Wegener granulomatosis (WG), either systemic WG with respiratory and renal involvement or limited WG with more restricted end-organ involvement. Positive results for pANCA are consistent with the diagnosis of autoimmune vasculitis including microscopic polyangiitis (MPA) or pauci-immune necrotizing glomerulonephritis. Sequential measurements of titers of cANCA may be useful to indicate the clinical course of patients with WG. Changes in titer of 2 or more serial dilutions are considered significant.(3) In patients with very low levels of cANCA, the immunofluorescent staining pattern may mimic the pANCA pattern. In patients with MPA, monitoring of disease activity may be performed by measuring MPO ANCA (MPO / Myeloperoxidase Antibodies, IgG, Serum).

Analytic Time

2 days

Day(s) and Time(s) Performed

Monday through Saturday; 11 a.m.

Clinical Reference

1. 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

2. Russell KA, Wiegert E, Schroeder DR, et al: Detection of anti-neutrophil cytoplasmic antibodies under actual clinical testing conditions. Clin Immunol 2002:103;196-203

3. Specks U, Homburger HA, DeRemee RA: Implications of cANCA testing for the classification of Wegener's Granulomatosis: performance of different detection systems. Adv Exp Med Biol 1993:336;65-70

Method Name

Indirect Immunofluorescence

Specimen Type

Serum


Additional Testing Requirements


When used for diagnosis, it is recommended that specific tests for proteinase 3 (PR3) ANCA and myeloperoxidase (MPO) ANCA be performed in addition to testing for cANCA and pANCA.(1) This panel of tests is available by ordering the VASC / Antineutrophil Cytoplasmic Antibodies Vasculitis Panel, Serum.



Specimen Required


Container/Tube:

Preferred: Serum gel

Acceptable: Red top

Specimen Volume: 0.8 mL


Specimen Minimum Volume

0.4 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Refrigerated (preferred) 14 days
  Frozen  14 days

Reference Values

Negative

If positive for antineutrophil cytoplasmic antibodies, results are titered.

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

86255

86256 (if appropriate)

LOINC Code Information

Test ID Test Order Name Order LOINC Value
ANCA Cytoplasmic Neutrophilic Ab, S 87427-1

 

Result ID Test Result Name Result LOINC Value
3114 c-ANCA 35279-9
3119 p-ANCA 17357-5

Forms

If not ordering electronically, complete, print, and send 1 of the following forms with the specimen:

-General Request (T239)

-Renal Diagnostics Test Request (T830)

Mayo Clinic Laboratories | Neurology Catalog Additional Information:

mml-Behavioral, mml-Cerebrovascular, mml-Epilepsy, mml-Headache, mml-Movement-Disorders, mml-Demyelinating-Diseases, mml-Neuroimmunology, mml-Neuromuscular, mml-Autonomic, mml-Pediatric, mml-Spinal-Cord