Managing Hypocomplement Urticarial Vasculitis: Treatment Options and Guidance - Internal Medicine

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Hypocomplementemic urticarial vasculitis syndrome (HUVS)


I have been diagnosed with low complement urticarial vasculitis for about six months.
My symptoms include skin rashes, weight loss, lower extremity vascular pain, and gum pain, along with peripheral neuropathy and dizziness.
Over the past six months, my test results showed low C3 levels ranging from 63 to 76 (normal range 90-180), normal C4, and C1q and C1c levels between 13 and 25.5 (positive if >10.8).
My white blood cell count is between 3300 and 5000, and my platelet count is between 120,000 and 150,000.
Liver function tests show GPT levels between 53 and 83, while other immune markers are normal.
I have been receiving treatment with one quinine tablet daily for six months and half a steroid tablet daily for about three months.
My symptoms fluctuate, but C3, C1q, and C1c levels remain abnormal.
How should low complement urticarial vasculitis be treated? Should I continue taking steroids? Thank you, Dr.
Gao.

Richard, 60~69 year old female. Ask Date: 2023/12/08

Dr. Gao Jiankai reply Internal Medicine


Hello, thank you for your question.
Here are my responses:
1.
How to treat hypocomplementemic urticarial vasculitis? --> Treatment will be arranged based on the severity of the condition.
If you only have skin manifestations, antihistamines may be effective; if there is associated arthritis, anti-inflammatory pain relievers can be added.
If the condition is more severe and antihistamines are not effective, corticosteroids will be used, and quinine may be added if necessary, as per your current prescription.
In cases that are even more severe, such as those affecting the kidneys, in addition to increasing the corticosteroid dosage, immunosuppressants will be utilized.
2.
Should I continue taking corticosteroids? --> It is recommended to continue taking them for now.
The timing for tapering or discontinuing medication is usually after clinical symptoms have been controlled for a period of time; typically, I would extend treatment for about a year before attempting to reduce or stop the medication.
This aspect can be discussed with your physician, as experiences may vary among different doctors.

Best regards from the Allergy, Immunology, and Rheumatology Department of Changhua Hospital, Ministry of Health and Welfare.

Reply Date: 2023/12/08

More Info


Managing hypocomplement urticarial vasculitis (HUV) can be quite challenging due to its complex nature and the variability in symptoms. HUV is characterized by recurrent urticarial lesions, often associated with low complement levels, particularly C3 and C4. Your symptoms, including skin rashes, weight loss, lower extremity pain, and peripheral neuropathy, indicate that the condition is significantly impacting your quality of life.


Treatment Options
1. Corticosteroids: You mentioned that you have been on a low dose of corticosteroids for about three months. Corticosteroids are often the first line of treatment for HUV due to their anti-inflammatory properties. They can help reduce the severity of symptoms and control the underlying inflammation. However, the decision to continue or adjust the dosage should be made in consultation with your healthcare provider, considering the potential side effects of long-term steroid use.

2. Immunosuppressive Agents: If corticosteroids alone are insufficient in managing your symptoms or if you experience significant side effects, your doctor may consider adding immunosuppressive medications. Drugs such as azathioprine, mycophenolate mofetil, or cyclophosphamide may be used to help control the immune response and reduce inflammation.

3. Antihistamines: Non-sedating antihistamines can be beneficial in managing urticarial symptoms. They can help alleviate itching and reduce the frequency of rash outbreaks. Some patients find that increasing the dose of antihistamines can provide additional relief.

4. Quinidine: You mentioned taking quinine, which is sometimes used for its anti-inflammatory properties, particularly in cases of chronic pain. However, its effectiveness in HUV is not well established, and it may not address the underlying complement deficiency.

5. Biologics: In more severe or refractory cases, biologic therapies targeting specific components of the immune system may be considered. Medications such as rituximab, which depletes B cells, or eculizumab, which inhibits complement activation, have shown promise in treating various forms of vasculitis, although their use in HUV specifically may require further investigation.

6. Lifestyle Modifications: Managing stress, maintaining a healthy diet, and avoiding known triggers (such as certain medications or foods) can also play a role in symptom management. Regular follow-ups with your healthcare provider are essential to monitor your condition and adjust treatment as necessary.


Monitoring and Follow-Up
Given that your complement levels (C3 and C1q) remain abnormal, it is crucial to have regular follow-ups with your healthcare provider. They may recommend periodic blood tests to monitor your complement levels and overall health. If your symptoms fluctuate, it may indicate that your treatment plan needs to be adjusted.


Conclusion
In summary, the management of hypocomplement urticarial vasculitis requires a tailored approach based on individual symptoms and response to treatment. Continuing corticosteroids may be necessary, but it is essential to discuss with your doctor whether to maintain, adjust, or explore additional treatment options. Collaboration with a specialist in rheumatology or immunology may provide further insights and management strategies. Your health and well-being are paramount, so ensure that you communicate openly with your healthcare team about your symptoms and treatment preferences.

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