From the Guidelines
The best treatment for urticarial vasculitis is a short tapering course of oral corticosteroids, such as prednisolone 50 mg daily for 3-4 weeks, as recommended by the British Journal of Dermatology guidelines 1. This approach is based on the guidelines for evaluation and management of urticaria in adults and children, which suggest that oral corticosteroids may shorten the duration of acute urticaria and are necessary for urticarial vasculitis and severe delayed pressure urticaria 1. Some key points to consider when treating urticarial vasculitis include:
- The use of oral corticosteroids, such as prednisolone, to reduce inflammation and control symptoms
- The importance of tailoring treatment to disease severity and underlying causes
- The need to avoid triggers like NSAIDs and monitor for systemic involvement affecting kidneys, lungs, or joints
- The goal of treatment is to control inflammation, which is driven by immune complex deposition in blood vessel walls, leading to the characteristic urticarial lesions that last longer than 24 hours and may leave residual hyperpigmentation or purpura. It is essential to note that long-term oral corticosteroids should not be used in chronic urticaria, except in very selected cases under regular specialist supervision, as stated in the guidelines 1.
From the Research
Treatment Options for Urticarial Vasculitis
- The treatment of urticarial vasculitis (UV) is challenging due to its variable response to available therapies 2, 3, 4.
- Oral corticosteroids are considered the first-line treatment for UV, but other agents such as H1 antihistamines, dapsone, colchicine, antimalarials, ciclosporin, and antileucotrienes have also been used 2, 3.
- Newer agents like omalizumab, an anti-IgE antibody, have shown efficacy in treating UV, particularly in cases refractory to conventional therapies 2, 5, 6, 4.
- A clinical decision-making algorithm for UV management has been proposed, which includes the use of second-generation H1-antihistamines, omalizumab, and cyclosporine A for patients with mild symptoms, and immunomodulatory therapy for those with more severe symptoms 6.
- Other treatment options that have been reported to be effective in UV include cyclophosphamide, tocilizumab, anti-interleukin (IL)-1 agents, and rituximab 5, 4.
Factors Influencing Treatment Choice
- The severity of skin and systemic involvement, as well as the underlying systemic disease, dictate the treatment approach for UV 5, 3, 4.
- Patients with hypocomplementemic UV tend to have more severe multi-organ involvement and may require more aggressive treatment 3.
- The presence of underlying diseases, such as systemic lupus erythematosus, cancer, or infection, may also influence the treatment choice 5, 3.
Efficacy of Treatment Options
- Corticosteroids are effective in treating skin symptoms in more than 80% of patients with UV, but their long-term use can lead to adverse effects 4.
- The addition of immunomodulatory or immunosuppressive agents can improve the efficacy of therapy and allow corticosteroid tapering 4.
- Biological agents, such as omalizumab, have shown promise in treating UV, particularly in cases refractory to conventional therapies 2, 5, 6, 4.