What is the treatment for IgA (Immunoglobulin A) vasculitis?

Treatment of IgA Vasculitis

The treatment of IgA vasculitis should be based on disease severity, with glucocorticoids as first-line therapy for mild to moderate disease and a combination of glucocorticoids with immunosuppressive agents (cyclophosphamide or rituximab) for severe, organ-threatening disease. 1

Disease Classification and Treatment Approach

Mild Disease (Non-Organ Threatening)

• Clinical presentation: Cutaneous purpura, arthralgia/arthritis, mild gastrointestinal symptoms
• Treatment:
  • Supportive care for self-limited disease
  • NSAIDs for joint pain
  • Glucocorticoids (prednisone 0.5-1 mg/kg/day) for persistent symptoms

Severe Disease (Organ-Threatening)

• Clinical presentation: Glomerulonephritis, alveolar hemorrhage, CNS involvement, mononeuritis multiplex, cardiac involvement, mesenteric ischemia
• Treatment:
  • High-dose glucocorticoids (prednisone 1 mg/kg/day, up to 80 mg/day)
  • PLUS immunosuppressive therapy:
    • Cyclophosphamide (2 mg/kg/day oral or 15 mg/kg IV pulse) OR
    • Rituximab (375 mg/m² weekly for 4 doses or 1,000 mg on days 1 and 15)

Specific Organ Involvement Treatment

Renal Involvement

• For proteinuria >1 g/day with eGFR ≥30 mL/min/1.73m²:

  • 6-month course of glucocorticoids
  • ACE inhibitor or ARB to achieve proteinuria <1 g/day
  • Target BP <125/75 mmHg 1

• For rapidly progressive glomerulonephritis (>50% crescents):

  • Cyclophosphamide + high-dose corticosteroids
  • Consider plasma exchange for severe disease 1

Gastrointestinal Involvement

• For mild symptoms: Supportive care
• For severe symptoms (severe abdominal pain, GI bleeding):
  • High-dose glucocorticoids
  • Consider immunosuppressive therapy if symptoms persist 2
  • Monitor for potential GI infections which may complicate management 3

Maintenance Treatment

After achieving remission:

• Continue treatment for at least 24 months
• Options include:
  • Azathioprine (2 mg/kg/day)
  • Methotrexate (15-25 mg/week)
  • Mycophenolate mofetil (2 g/day)
  • Rituximab (for maintenance after rituximab induction) 1

Monitoring and Prevention

• Regular urinalysis at each visit to screen for infection, renal relapse, or treatment response

• Periodic assessment (every 1-3 months) of:

  • Inflammatory markers
  • Renal function
  • Complete blood count
  • Liver function 1

• For patients on cyclophosphamide:

  • Monitor for leukopenia and adjust dosage as needed
  • Investigate persistent unexplained hematuria (risk of bladder cancer)
  • Consider prophylaxis against Pneumocystis jirovecii with trimethoprim/sulfamethoxazole 1

• For patients on rituximab:

  • Test serum immunoglobulin levels prior to each course
  • Monitor for hypogammaglobulinemia in patients with recurrent infections 1

Special Considerations

• Adult-onset IgA vasculitis typically has a more severe course than pediatric cases, with higher risk of evolving into end-stage renal disease 4
• Treatment decisions should not be based solely on laboratory markers but on clinical manifestations and organ involvement
• Cardiovascular risk assessment should be performed periodically 1

Treatment for Refractory Disease

For patients not responding to standard therapy:

• Consider alternative immunosuppressants:
  • Mycophenolate mofetil
  • Calcineurin inhibitors (cyclosporine A, tacrolimus)
  • Leflunomide
• IVIG and plasma exchange may be useful in difficult and life-threatening situations 5

While IgA vasculitis can be self-limiting in many cases, prompt recognition and appropriate treatment of severe manifestations are crucial to prevent long-term complications, particularly renal damage which can lead to end-stage renal disease in adults.