To help detect, diagnose, and sometimes monitor certain forms of systemic vasculitis (an autoimmune disorder that causes inflammation of blood vessels)
To help distinguish between Crohn disease (CD) and ulcerative colitis (UC), the two most common types of inflammatory bowel disease (IBD); as an adjunct to other IBD testing
When you have symptoms such as fever, muscle aches, and weight loss or impaired kidney or lung function that your healthcare practitioner thinks may be due to a vascular autoimmune disorder
When you have symptoms such as persistent or intermittent diarrhea and abdominal pain that your healthcare practitioner suspects may be due to an IBD; when your healthcare practitioner wants to distinguish between CD and UC
A blood sample drawn from a vein in your arm
Antineutrophil cytoplasmic antibodies (ANCA) are autoantibodies produced by the immune system that mistakenly target and attack specific proteins within neutrophils (a type of white blood cell). ANCA testing detects and measures the amount of these autoantibodies in the blood. Two of the most common ANCAs are the autoantibodies that target the proteins myeloperoxidase (MPO) and proteinase 3 (PR3). These are called pANCAs and cANCAs, respectively.
There are two types of ANCA tests:
The first type is called Indirect Immunofluorescence (IIF). This uses neutrophils fixed onto a slide. For the test, serum from your blood sample is mixed with the neutrophils on the slide and any ANCAs in the sample attach to the neutrophil proteins. Treatment of the slide with a fluorochrome-stained antibody reacts with any ANCA present. This produces a pattern of fluorescence that can be seen under a microscope. The pattern may be identified as cytoplasmic (cANCA), perinuclear (pANCA), or atypical ANCA (X-ANCA).
Alternatively, the laboratory may test for antibodies to myeloperoxidase or to proteinase 3 directly using an ELISA assay.
A combination of both fluorescence and ELISA tests are often done when testing suspected cases of vasculitis.
ANCA may be present in several autoimmune disorders that cause inflammation, tissue damage, and organ failure:
(For more information on these specific conditions, see the article on Vasculitis).
cANCA/PR3 antibodies are most frequently seen in granulomatosis with polyangiitis and pANCA/ MPO antibodies are most often associated with microscopic polyangiitis. However, both may be seen in all three types with varying degrees of reactivity.
Tests for antineutrophil cytoplasmic antibodies (ANCA) may be used to:
Some laboratories will perform all three tests, ANCA, MPO and PR3, as a panel while others will perform MPO and PR3 only if an initial ANCA test is positive.
An ANCA test and/or tests for MPO and PR3 are ordered when you have signs and symptoms that suggest systemic autoimmune vasculitis. Early in the disease, symptoms may be vague or nonspecific, such as fever, fatigue, weight loss, muscle and/or joint aches, and night sweats. As the disease progresses, damage to blood vessels throughout the body may cause signs and symptoms associated with complications involving various tissues and organs. A few examples include:
Testing may also be performed periodically when you are being treated for autoimmune vasculitis.
Inflammatory Bowel Disease
An ANCA test may be ordered with a test for anti-Saccharomyces cerevisiae antibodies (ASCA) when you have signs and symptoms that suggest inflammatory bowel disease and your healthcare practitioner is attempting to distinguish between Crohn disease and ulcerative colitis.
Symptoms of an IBD may include:
Results of ANCA tests must be interpreted carefully, taking several factors into account. A healthcare practitioner will consider your signs and symptoms in addition to results of the laboratory tests and other types of tests, such as imaging studies.
Positive test results for ANCA, PR3, and/or MPO help support a diagnosis of systemic autoimmune vasculitis and distinguish between different types of vasculitis. However, to confirm a diagnosis, a biopsy of an affected site is often required.
Negative ANCA tests results mean it is unlikely that your symptoms are due to an autoimmune vasculitis.
For a positive result on the indirect immunofluorescence microscopy method, different ANCA patterns may be seen:
If an ANCA test result is positive, then an additional step may be performed to determine the amount of antibody present. This is called a titer. To determine the titer, a serum sample is diluted in steps and each dilution is tested for the presence of the antibody. The greatest dilution at which the antibody can be detected is the titer. For example, if a serum tests positive after being diluted 64-fold, the titer is 1:64. The higher the titer, the more antibody is present in the blood.
ANCA levels can change over time and may sometimes be used in a general way to monitor disease activity and/or response to therapy. However, in many individuals, titer levels may not correlate with the extent of disease activity.
The following table shows results that may be seen in some vasculitis conditions.
|Condition||% of patients with cANCA pattern (PR3 antibodies)||% of patients with pANCA pattern (MPO antibodies)|
|Granulomatosis with polyangiitis (Wegener granulomatosis)||90% in active disease, 60-70% no active disease||Less than 10%|
|Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome)||Rare||50-80%|
Inflammatory Bowel Disease
In cases of suspected inflammatory bowel disease (IBD):
Additional tests that may be performed to aid in diagnosis include erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP) to check for inflammation, complete blood count (CBC) to measure and evaluate white and red blood cells, and urinalysis, blood urea nitrogen (BUN), and creatinine to evaluate kidney function. For some patients, tests for viruses such as hepatitis or cytomegalovirus may be ordered.
Since the symptoms associated with vasculitis and inflammatory bowel disease may be seen with a number of conditions, other tests are frequently performed prior to or along with ANCA testing to rule out other causes for the symptoms.
In most cases, a biopsy of an affected blood vessel is necessary to confirm a diagnosis of autoimmune vasculitis.
Levels may fluctuate, but once you develop an autoantibody, you will continue to have it.
ANCA testing requires specialized equipment and careful interpretation by trained professionals. Your sample will need to be sent to a clinical laboratory that performs these tests and may be sent to a reference laboratory. Depending on the lab, it may take a few days to a week for results to be available.
Yes. These conditions include rheumatoid arthritis, systemic lupus erythematosus (SLE), lung conditions, autoimmune hepatitis, use of certain drugs, and infections involving the heart (endocarditis) or the respiratory system.
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