What is the management approach for leukocytoclastic vasculitis?

Management of Leukocytoclastic Vasculitis

The management of leukocytoclastic vasculitis (LCV) requires a tailored approach based on disease severity, with organ-threatening or life-threatening disease requiring high-dose glucocorticoids plus either cyclophosphamide or rituximab as first-line therapy. 1

Initial Assessment and Classification

• Determine if LCV is:

  • Skin-limited
  • Systemic with organ involvement
  • ANCA-associated
  • Secondary to underlying causes (medications, infections, malignancy)

• Essential diagnostic workup:

  • Skin biopsy with immunofluorescence (gold standard)
  • Complete blood count
  • Renal function tests and urinalysis
  • Serological tests (ANCA, ANA, complement levels)
  • IgA staining in biopsy specimens

Treatment Algorithm Based on Disease Severity

1. Organ-Threatening or Life-Threatening Disease

• First-line therapy:

  • High-dose glucocorticoids (40-60 mg/day prednisone equivalent)
  • PLUS either:
    • Cyclophosphamide (oral: 2 mg/kg/day, max 200 mg/day; or IV: 0.6 mg/m² every 2 weeks initially) 1
    • OR Rituximab (375 mg/m² once weekly for four infusions) 1

• Consider plasma exchange for patients with rapidly progressive renal failure (serum creatinine >300 µmol/L due to active glomerulonephritis) 2

• Glucocorticoid tapering schedule:

  • Reduce to 15-20 mg/day within 2-3 months
  • Aim for ≤5 mg/day after 1 year 1

2. Non-Organ-Threatening Disease

• First-line therapy:
  • Glucocorticoids plus rituximab 2
  • Alternatives: Methotrexate or mycophenolate mofetil 2

3. Skin-Limited LCV

• First-line therapy:
  • Identify and remove triggers (medications, infections)
  • Rest and elevation of affected areas
  • Low-dose corticosteroids
  • Colchicine (first-line for chronic/relapsing cases) 3
  • Dapsone (second-line for chronic/relapsing cases) 3

Maintenance Therapy

• After achieving remission, transition to:
  • Azathioprine or methotrexate 1
  • Consider rituximab for maintenance in selected cases 1
  • Continue maintenance therapy for at least 24 months 2

Prevention of Treatment Complications

• Pneumocystis jirovecii pneumonia prophylaxis:

  • Trimethoprim-sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) for patients on cyclophosphamide, rituximab, or high-dose glucocorticoids 1

• Monitoring for cyclophosphamide toxicity:

  • Regular blood counts
  • Urinalysis (risk of hemorrhagic cystitis)
  • Consider mesna for bladder protection 2

• Monitoring for rituximab:

  • Serum immunoglobulin concentrations prior to each course 1
  • Hepatitis B screening before initiation

Management of Relapses

• For major relapse:
  • Reinstitute or escalate glucocorticoid therapy
  • Consider switching from cyclophosphamide to rituximab or vice versa 2
  • Optimize immunosuppressive therapy

Special Considerations

• IgA-dominant LCV (Henoch-Schönlein purpura):

  • Higher risk of systemic involvement
  • More aggressive treatment may be needed 3

• Cryoglobulinemic vasculitis:

  • Treat underlying disease (often hepatitis C or plasmocytoma)
  • Consider plasmapheresis in severe cases 4

• Urticarial vasculitis:

  • Usually responsive to dapsone or colchicine 4

Follow-up and Monitoring

• Every 1-3 months during the first year
• Every 3-6 months thereafter
• Monitor for disease activity and treatment-related adverse effects 1
• Patients with AAV should be managed in close collaboration with, or at, centers of expertise 2

Vascular Surgical Considerations

• Vascular surgery consultation for:
  • Critical limb ischemia
  • Arterial vessel dissection
  • Aneurysm formation at high risk for rupture 1
• Delay surgical intervention until disease is quiescent when possible 1