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Test ID: ANCA Cytoplasmic Neutrophil Antibodies, Serum

Reporting Name

Cytoplasmic Neutrophilic Ab, S

Useful 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 days

Day(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

Serum


Additional 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)

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