What is the treatment for leukocytoclastic vasculitis?

Treatment of Leukocytoclastic Vasculitis

For leukocytoclastic vasculitis (LCV), treatment should be based on disease severity, with skin-limited disease requiring less aggressive therapy than systemic disease, and may include corticosteroids, colchicine, dapsone, or immunosuppressants depending on severity and organ involvement. 1

Disease Classification and Assessment

Disease severity in LCV is categorized according to the European Vasculitis Study Group (EUVAS) classification into:

• Localized
• Early systemic
• Generalized
• Severe
• Refractory

This classification guides treatment decisions and helps determine appropriate therapy 1.

Treatment Algorithm Based on Disease Severity

1. Skin-Limited LCV

• First-line options:

  • Symptomatic treatment with rest and compression stockings
  • Low-dose corticosteroids for symptomatic relief
  • Colchicine as first-line therapy for chronic/relapsing cases 1, 2
  • Dapsone as second-line therapy 2

• For persistent or recurrent skin-limited LCV:

  • Methotrexate (oral or parenteral) with glucocorticoids 1
  • Mycophenolate mofetil as an alternative 1

2. Early Systemic or Generalized LCV

• Treatment approach:
  • Higher-dose glucocorticoids (prednisolone 40-60 mg/day)
  • Consider adding immunosuppressants based on severity 1

3. Severe or Life-Threatening LCV

• Immediate treatment required:

  • High-dose glucocorticoids (prednisolone 50-75 mg/day)
  • Combined with either:
    • Rituximab (preferred for relapsing disease)
    • Cyclophosphamide (for severe manifestations) 1

• For rapidly progressive glomerulonephritis or diffuse alveolar hemorrhage:

  • Consider plasma exchange
  • Avacopan may be considered to reduce glucocorticoid exposure 1

Special Considerations

IgA-Dominant LCV (Henoch-Schönlein Purpura)

• More likely to have systemic involvement
• May require more aggressive therapy, especially in adults 2, 3
• Corticosteroids may help prevent severe renal insufficiency in children, but evidence is insufficient for adults 1, 2

Medication-Induced LCV

• Discontinuation of the culprit drug is usually curative 4
• Symptomatic treatment may be needed during resolution

Cryoglobulinemic Vasculitis

• Treat underlying disease (often hepatitis C or plasmacytoma)
• Consider plasmapheresis in severe cases 5

Maintenance Therapy

After achieving remission:

• Rituximab is the preferred maintenance therapy
• Alternatives include azathioprine, methotrexate, or mycophenolate mofetil
• Continue maintenance therapy for 24-48 months following remission 1

Monitoring and Follow-up

• Regular structured clinical assessment rather than ANCA testing alone
• Monitor immunoglobulin levels prior to each rituximab course
• For patients on cyclophosphamide:
  • Monitor for unexplained hematuria
  • Consider prophylaxis against Pneumocystis jiroveci with trimethoprim/sulfamethoxazole 1

Treatment Pitfalls and Caveats

1. Don't delay aggressive treatment in organ or life-threatening disease
2. Don't rely solely on corticosteroids for severe systemic vasculitis, as they may not prevent severe renal insufficiency in adults 1
3. Don't overlook underlying causes - infections, medications, and malignancies should be identified and addressed 4
4. Don't forget multidisciplinary care - collaboration between rheumatologists, dermatologists, and other specialists based on organ involvement is essential 1
5. Don't underestimate the need for long-term follow-up - relapses may occur years after remission 1

Refractory Cases

For cases not responding to standard therapy:

• Refer to a specialized vasculitis center
• Consider alternative immunosuppressants or biological therapies like TNF-α inhibitors
• Combination therapy with glucocorticoids and methotrexate or mycophenolate mofetil may be effective 1