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HelpTest ID: ANCA Cytoplasmic Neutrophil Antibodies, Serum
Reporting Name
Cytoplasmic Neutrophilic Ab, SUseful For
Evaluating patients suspected of having ANCA-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis)
Clinical Information
Antineutrophil cytoplasmic antibodies (ANCA) can occur in patients with small blood vessel vasculitis including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), or eosinophilic granulomatosis with polyangiitis (EGPA), collectively referred to as ANCA-associated vasculitis (AAV).(2) Detection of ANCA is a well-established diagnostic test for the evaluation of patients suspected of having AAV.(3) ANCA react with enzymes in the cytoplasmic granules of human neutrophils including proteinase 3 (PR3), myeloperoxidase (MPO), elastase, and cathepsin G amongst others. Of these, PR3-ANCA and MPO-ANCA are the best characterized in AAV. Antibodies to PR3-ANCA occur in patients with GPA and produce a characteristic pattern of granular cytoplasmic fluorescence on ethanol-fixed neutrophils called the cANCA pattern. Antibodies to MPO-ANCA occur predominately in patients with MPA and produce a pattern of perinuclear cytoplasmic fluorescence on ethanol-fixed neutrophils called the pANCA pattern.(4) EPGA may be pANCA positive with reactivity to MPO-ANCA or negative for ANCA. The pANCA pattern may also be observed in patients with inflammatory bowel disease, predominantly ulcerative colitis, usually in the absence of detectable MPO-ANCA reactivity.
Interpretation
Positive results for antineutrophil cytoplasmic antibodies (ANCA) demonstrate two main patterns namely; cytoplasmic (cANCA) and perinuclear (pANCA) in a compendium of small vessel vasculitis collectively referred to as ANCA-associated vasculitis (AAV) that includes granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis.
Negative ANCA results do not rule out a diagnosis of AAV or irritable bowel disease.
Report Available
2 to 4 daysDay(s) Performed
Monday through Saturday
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. Kitching AR, Anders HJ, Basu N, et al: ANCA-associated vasculitis. Nat Rev Dis Primers. 2020 Aug;6(1);71
3. Ramponi G, Folci M, De Santis M, et al: The biology, pathogenetic role, clinical implications, and open issues of serum anti-neutrophil cytoplasmic antibodies. Autoimmun Rev. 2021 Mar;20(3):102759
4. Guchelaar NAD, Waling MM, Adhin AA, et al: The value of anti-neutrophil cytoplasmic antibodies (ANCA) testing for the diagnosis of ANCA-associated vasculitis, a systematic review and meta-analysis. Autoimmun Rev. 2021 Jan:20;102716
Method Name
Indirect Immunofluorescence
Specimen Type
SerumAdditional Testing Requirements
When used for diagnosis, it is recommended that specific tests for proteinase 3, antineutrophil cytoplasmic antibodies (ANCA), and myeloperoxidase 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 US Food and Drug Administration.CPT Code Information
86036
86037-Titer (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)
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