From the Guidelines
The treatment of urticarial vasculitis should prioritize short courses of oral corticosteroids, such as prednisolone 50 mg daily for 3 days, which may be tapered over 3-4 weeks for severe cases or those with significant inflammation 1.
Key Considerations
- The primary goal is to control inflammation and address underlying causes, with corticosteroids being a cornerstone in managing acute and severe presentations.
- For mild cases, nonsteroidal anti-inflammatory drugs (NSAIDs) like naproxen may be considered, but their efficacy in urticarial vasculitis is less clear compared to corticosteroids.
- The use of antihistamines, such as cetirizine or hydroxyzine, may help manage urticarial symptoms but are often insufficient on their own for urticarial vasculitis.
- In cases where urticarial vasculitis is recurrent or steroid-dependent, immunosuppressive agents may be necessary, but the choice of agent should be guided by the severity of the disease, potential side effects, and the presence of any underlying conditions.
Treatment Approach
- Short courses of oral corticosteroids are recommended for severe acute urticaria or angio-oedema affecting the mouth, with more prolonged treatment considered for delayed pressure urticaria or urticarial vasculitis 1.
- The dose and duration of corticosteroids should be tailored to the individual's response and the severity of the disease, with a gradual taper to minimize side effects.
- Regular monitoring for medication side effects and disease activity is crucial, including laboratory tests to assess for systemic involvement.
- Treatment of any identified underlying conditions is essential and should be managed concurrently with urticarial vasculitis therapy.
Important Considerations
- The quality of evidence for specific treatments in urticarial vasculitis may vary, and clinical decisions should be based on the most recent and highest quality studies available.
- Given the potential for significant morbidity and impact on quality of life, a cautious approach that prioritizes effective control of symptoms while minimizing long-term side effects is recommended.
From the Research
Treatment Options for Urticarial Vasculitis
- Corticosteroids are effective for the treatment of skin symptoms in more than 80% of patients with UV, but their long-term administration can lead to potentially serious adverse effects 2
- The addition of immunomodulatory or immunosuppressive agents often allows corticosteroid tapering and improves the efficacy of therapy 2
- Biologicals, including omalizumab, as well as cyclophosphamide, dapsone, mycophenolate mofetil, plasmapheresis, colchicine, hydroxychloroquine, intravenous immunoglobulin, nonsteroidal anti-inflammatory drugs, and cyclosporine, can be effective for both skin and systemic symptoms in patients with UV 2, 3, 4
- Antihistamines, systemic corticosteroids, omalizumab, cyclophosphamide, tocilizumab, anti-interleukin (IL)-1 agents, and rituximab were shown to have the highest success rates in treating UV 3
- Rituximab, dapsone, and MMF were related to long-lasting treatment-free responses 3
Treatment Algorithms and Guidelines
- A clinical decision-making algorithm for UV management based on the Urticarial Vasculitis Activity Score assessed for 7 days (UVAS7) has been proposed 5
- Patients with occasional UV-like urticarial lesions and patients with UV with skin-limited manifestations and/or mild arthralgia/malaise can be initially treated using the step-wise algorithm for chronic urticaria including second-generation H1-antihistamines, omalizumab, and cyclosporine A 5
- Patients with UV with more severe symptoms may require a multidisciplinary approach, particularly if underlying diseases are present 5
Challenges in Managing Urticarial Vasculitis
- The greatest perceived challenges in the management of UV were the limited efficacy of drugs and the absence of clinical guidelines and treatment algorithms 6
- International consensus-based recommendations for the classification of UV and the diagnostic workup and treatment, as well as prospective studies evaluating potentially safe and effective drugs for the treatment of UV, are necessary 2, 6