What is the treatment for urticarial vasculitis?

Treatment for Urticarial Vasculitis

Treatment for urticarial vasculitis should follow a stepwise approach based on disease severity, with systemic corticosteroids being the most effective first-line therapy for most patients, achieving response rates over 80%. 1

Disease Classification and Assessment

Before initiating treatment, classify the patient's condition:

1. Skin-limited UV vs. Systemic UV

   • Assess for extracutaneous manifestations (present in up to 81% of patients) 2
   • Check for hypocomplementemia (present in 11% of UV patients) 2
   • Use the Urticarial Vasculitis Activity Score (UVAS7) to quantify severity 3

2. Diagnostic Confirmation

   • Skin biopsy showing leukocytoclastic vasculitis is essential for diagnosis
   • Dermoscopy may help differentiate UV from chronic spontaneous urticaria (purpuric patches and globules favor UV diagnosis) 4
   • Rule out underlying conditions (autoimmune diseases, infections, malignancy, drug reactions) 4

Treatment Algorithm

Mild UV (Skin-limited, UVAS7 ≤7)

1. First-line:

   • Second-generation H1-antihistamines at standard doses 5
   • Note: Unlike chronic urticaria, antihistamines alone are typically ineffective in most UV patients 1

2. Second-line:

   • Short course of systemic corticosteroids (prednisone 0.5-1 mg/kg/day) 5, 1
   • Taper once symptoms resolve to grade 1 or less

3. Third-line (if inadequate response):

   • Consider adding immunomodulatory agents to allow steroid tapering:
     • Dapsone
     • Hydroxychloroquine
     • Colchicine (0.5 mg three times daily) 6

Moderate to Severe UV (UVAS7 >7 or Hypocomplementemic UV)

1. First-line:

   • Systemic corticosteroids (prednisone 0.5-1 mg/kg/day) 5, 1

2. Second-line (steroid-sparing agents):

   • Cyclosporine (up to 5mg/kg body weight) 5, 1
   • Mycophenolate mofetil
   • Dapsone
   • Hydroxychloroquine

3. Third-line (refractory cases):

   • Biologics:
     • Omalizumab (300mg subcutaneously every 4 weeks) 5, 4
     • Rituximab (associated with long-lasting treatment-free responses) 4
     • IL-1 inhibitors 4
   • Cyclophosphamide (for severe systemic disease) 1, 4
   • Intravenous immunoglobulin 1
   • Plasmapheresis (for severe cases) 1

Special Considerations

1. Monitoring:

   • For cyclosporine: Check blood pressure and renal function every 6 weeks 5
   • For all patients: Regular assessment of disease activity using validated tools

2. Ineffective Treatments to Avoid:

   • Montelukast
   • H2-antihistamines
   • Tranexamic acid
   • Pentoxifylline 1

3. Associated Conditions:

   • If UV is associated with underlying conditions (SLE, infections, VEXAS syndrome), treat the primary disease 4
   • Recent reports link UV to COVID-19 and COVID-19 vaccines 4

4. Prognosis:

   • Hypocomplementemic UV has 5-year and 10-year survival rates of 92% and 83%, respectively 4
   • Main causes of mortality: COPD, septicemia, and end-stage renal disease 4

Treatment Efficacy

The most recent comprehensive review indicates that:

• Corticosteroids are effective in >80% of UV patients 1
• Rituximab, dapsone, and mycophenolate mofetil provide the longest treatment-free remissions 4
• Omalizumab, cyclophosphamide, and IL-1 inhibitors show high success rates in refractory cases 4

It's important to note that the evidence for all UV treatments is limited, with most recommendations based on case reports and retrospective studies rather than randomized controlled trials 1, 3.