Overview
The C-ANCA test is a blood test that helps detect autoantibodies (abnormal immune proteins) that mistakenly attack the body’s own white blood cells.
C-ANCA stands for Cytoplasmic Anti-Neutrophil Cytoplasmic Antibody, and its detection is especially important in diagnosing vasculitis — a group of diseases that cause inflammation of blood vessels.
Among these, C-ANCA is most strongly linked to a serious autoimmune disease called Granulomatosis with Polyangiitis (GPA) (previously known as Wegener’s Granulomatosis). This condition causes inflammation in the small- and medium-sized blood vessels, often affecting the lungs, kidneys, sinuses, and other organs.
What is C-ANCA?
C-ANCA is an autoantibody — a harmful antibody made by the immune system that mistakenly attacks proteinase 3 (PR3), a normal protein found inside neutrophils (a type of white blood cell).
When these antibodies target PR3, they cause neutrophil activation, leading to inflammation and destruction of blood vessel walls.
This immune reaction damages tissues and organs, resulting in vasculitis and a variety of symptoms depending on the affected area.
The C-ANCA Test is Mainly Used To:
- Help diagnose autoimmune vasculitis, especially Granulomatosis with Polyangiitis (GPA)
- Monitor disease activity and treatment response
- Differentiate GPA from other similar conditions like Microscopic Polyangiitis (MPA) or P-ANCA–positive vasculitis
The C-ANCA test is often ordered together with P-ANCA testing, ESR (erythrocyte sedimentation rate), CRP (C-reactive protein), and kidney function tests for a full picture of inflammation and organ involvement.
Where is C-ANCA Produced in the Body?
C-ANCA antibodies are produced by B-cells, a type of white blood cell that is part of the body’s immune system.
In autoimmune vasculitis, the immune system becomes overactive and starts attacking the body’s own cells.
Mechanism:
- The B-cells create antibodies against proteinase 3 (PR3).
- PR3 is found in the cytoplasm of neutrophils, which are white blood cells responsible for fighting infections.
- When these antibodies bind to PR3, they cause neutrophil activation, releasing inflammatory chemicals that damage blood vessels.
This process results in vasculitis, which can affect multiple organs such as the lungs, kidneys, sinuses, eyes, and skin.
Main Functions and Importance of C-ANCA Test
C-ANCA antibodies themselves have no useful role — they are harmful autoantibodies. Their presence in blood indicates that the immune system is attacking healthy blood vessels, leading to tissue injury.
Importance of the C-ANCA Test:
- Helps diagnose Granulomatosis with Polyangiitis (GPA), a potentially life-threatening condition if untreated.
- Aids in monitoring disease activity and response to therapy (C-ANCA levels often fall as treatment works).
- Helps differentiate autoimmune vasculitis from other causes of inflammation or infection.
- Supports early detection before major organ damage occurs.
In short, this test is an essential diagnostic tool for doctors managing patients with systemic vasculitis.
Causes of Low or Negative C-ANCA Levels
A negative or low C-ANCA result means that no C-ANCA antibodies were found in the blood. This is usually a normal and healthy result.
Possible Reasons for Low/Negative C-ANCA:
- No autoimmune vasculitis or active inflammation.
- Disease remission (condition under control after treatment).
- Early or mild disease where antibodies haven’t yet developed.
- Other types of vasculitis that are not C-ANCA related (for example, P-ANCA–associated vasculitis).
Symptoms of Low/Negative C-ANCA Levels:
There are no direct symptoms caused by a negative result — it simply indicates absence of disease activity.
However, if vasculitis symptoms are still present, doctors may order P-ANCA testing, biopsy, or imaging tests to confirm the diagnosis.
Causes of High or Positive C-ANCA Levels
A positive C-ANCA result usually indicates the presence of autoimmune vasculitis. The test is highly specific for Granulomatosis with Polyangiitis (GPA) but can also be positive in a few other conditions.
Common Causes of High/Positive C-ANCA:
- Granulomatosis with Polyangiitis (GPA) – the most specific and common cause.
- Microscopic Polyangiitis (MPA) – less common and may also show P-ANCA positivity.
- Other autoimmune diseases (rarely).
- Certain infections or medications (very rare, transient elevations).
Symptoms of High C-ANCA Levels
C-ANCA antibodies themselves don’t cause symptoms — the symptoms result from the inflammation and blood vessel damage they trigger.
People with GPA (Wegener’s disease) and high C-ANCA may experience symptoms involving different organs:
Common Symptoms Include:
1. ENT (Ear, Nose, Throat):
- Chronic sinusitis or nasal congestion
- Frequent nosebleeds
- Nasal crusting, ulcers, or saddle-nose deformity
2. Lungs:
- Cough, chest pain, or shortness of breath
- Blood in sputum (hemoptysis)
3. Kidneys:
- Blood in urine
- Swelling in legs or around eyes
- High blood pressure
4. General / Systemic Symptoms:
- Fatigue and fever
- Weight loss and joint pain
- Skin rashes or ulcers
Symptoms depend on which organs are affected — and early diagnosis is crucial to prevent permanent damage.
Reference Ranges
| Result | Interpretation |
|---|---|
| Negative | No C-ANCA detected – Normal result |
| Positive (C-ANCA with PR3) | Suggests Granulomatosis with Polyangiitis (GPA) or related vasculitis |
Note: Test interpretation should always be correlated with clinical findings and other lab results (such as ESR, CRP, or biopsy).
Sample Type and Test Details
- Sample Type: Serum (blood sample)
- Tube Type: Red Top (Plain Tube or Serum Separator Tube)
- Fasting: Not required
- Timing: Can be done at any time of the day
The blood sample is analyzed using immunofluorescence or ELISA methods to detect antibodies against PR3.
Test Preparation
- No fasting or dietary restrictions are needed.
- Inform your doctor if you are on immunosuppressive drugs, steroids, or antibiotics, as they may influence results.
- If symptoms suggest vasculitis but the test is negative, follow-up tests (like P-ANCA or PR3/MPO ELISA) may be recommended.
When to Consult a Doctor
You should see a doctor if you experience:
- Unexplained sinus problems, nasal bleeding, or ulcers
- Persistent cough, chest pain, or breathing difficulty
- Blood in urine or swelling of legs/face
- Fever, fatigue, or joint pain without a known cause
These may be early signs of vasculitis, and early testing can prevent organ damage.
Important Word Explanations
- Autoantibody: A harmful antibody that attacks your own body’s tissues.
- Neutrophils: A type of white blood cell that fights infections.
- Cytoplasm: The inner part of a cell where C-ANCA binds to PR3 protein.
- Proteinase 3 (PR3): A protein in neutrophils targeted by C-ANCA.
- Vasculitis: Inflammation of blood vessels that can affect any organ.
- Granulomatosis with Polyangiitis (GPA): Autoimmune disease causing blood vessel inflammation.
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