Elevated Atypical p-ANCA Levels Despite Improved Vasculitis Symptoms

Elevated ANCA levels in vasculitis?

Hello Doctor, I previously had unexplained red spots on both of my calves.
After consulting a physician, I was diagnosed with vasculitis.
Over the course of about three months, I took antihistamines, corticosteroids, and colchicine, and my symptoms improved significantly.
However, during a recent check-up, I found that my Atypical p-ANCA level has increased to 100 (while c-ANCA and p-ANCA levels are normal).
Could you explain why my symptoms have improved but the levels have increased? What other diseases could this indicate? What should I be aware of?

Min, 60~69 year old female. Ask Date: 2022/01/12

Dr. Gao Jiankai reply Internal Medicine

Hello, thank you for your inquiry.
Atypical p-ANCA is less specific and may not necessarily correlate with disease activity.
If there are no significant symptoms, monitoring is sufficient.
It is recommended to maintain a regular lifestyle and incorporate appropriate exercise to stabilize immune function.
The Allergy, Immunology, and Rheumatology Department of Changhua Hospital, Ministry of Health and Welfare, cares about you.

Reply Date: 2022/01/12

More Info

Elevated atypical p-ANCA levels despite symptomatic improvement in vasculitis can be perplexing and may raise several questions regarding the underlying pathophysiology and the potential implications for your health. To understand this phenomenon, it is essential to consider the role of anti-neutrophil cytoplasmic antibodies (ANCA) in the context of vasculitis and other autoimmune conditions.

ANCA are autoantibodies that target proteins in neutrophils, and their presence is often associated with various forms of vasculitis, particularly those affecting small to medium-sized blood vessels. The two main types of ANCA are c-ANCA (cytoplasmic ANCA) and p-ANCA (perinuclear ANCA). Atypical p-ANCA refers to a subset of p-ANCA that does not fit the typical patterns seen in classic vasculitis, and its clinical significance can vary.

In your case, the improvement in symptoms suggests that the treatment regimen, which included antihistamines, corticosteroids, and colchicine, has been effective in managing the inflammatory process associated with your vasculitis. However, the rise in atypical p-ANCA levels could indicate several possibilities:
1. Residual Immune Activation: Even though your symptoms have improved, the immune system may still be in a state of heightened activity, leading to the production of atypical p-ANCA. This can occur as the body continues to respond to underlying triggers, which may not be entirely eliminated.

2. Different Disease Mechanisms: The elevation of atypical p-ANCA could suggest that there are other underlying autoimmune processes at play. Some patients may have overlapping autoimmune conditions, and the presence of atypical p-ANCA could be indicative of a different or additional pathology that requires further investigation.

3. Laboratory Variability: It is also important to consider that laboratory tests can have variability. Factors such as the timing of the test, the specific assay used, and even the patient's current medications can influence the results. It may be beneficial to repeat the test or consult with a specialist in autoimmune diseases for further evaluation.

4. Monitoring for Other Conditions: Elevated atypical p-ANCA levels can be associated with other conditions, including inflammatory bowel disease, primary sclerosing cholangitis, or even certain infections. It is crucial to monitor for any new symptoms or changes in your health that could indicate the development of another condition.

Given these considerations, it is advisable to discuss your elevated atypical p-ANCA levels with your healthcare provider. They may recommend additional testing, such as imaging studies or further serological tests, to assess for other potential autoimmune diseases or complications. It is also essential to maintain regular follow-ups to monitor your symptoms and any changes in your laboratory results.

In summary, while the improvement in your symptoms is a positive sign, the rise in atypical p-ANCA levels warrants further investigation. Understanding the broader context of your health, including any other symptoms or changes, will be critical in determining the next steps in your management. Your healthcare provider can help guide you through this process and ensure that you receive appropriate care tailored to your specific situation.

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