What is the management approach for recurrent IgA (Immunoglobulin A) vasculitis?

Management of Recurrent IgA Vasculitis

For recurrent IgA vasculitis, rituximab is the recommended treatment of choice, particularly for patients with relapsing disease, as it effectively reduces relapse frequency and achieves long-term remission while allowing for reduction in glucocorticoid exposure.

Assessment of Disease Severity

First, determine the severity of the recurrent IgA vasculitis:

• Organ-threatening/life-threatening manifestations:

  • Severe nephritis with declining renal function
  • Severe gastrointestinal involvement with bleeding
  • Pulmonary hemorrhage
  • Central nervous system involvement

• Non-organ-threatening manifestations:

  • Persistent cutaneous lesions
  • Mild arthralgia/arthritis
  • Mild gastrointestinal symptoms

Treatment Algorithm for Recurrent IgA Vasculitis

For Severe Recurrent Disease (Organ-Threatening):

1. First-line therapy:

   • Combination of high-dose glucocorticoids (prednisolone 50-75 mg/day) with rituximab 1
   • Rituximab is preferred over cyclophosphamide in relapsing disease 1
   • Rituximab dosing options:
     • 375 mg/m² weekly for 4 weeks 1, or
     • 1000 mg given twice, 2 weeks apart

2. Maintenance therapy after remission:

   • Rituximab maintenance using either:
     • MAINRITSAN scheme: 500 mg × 2 at complete remission, and 500 mg at months 6,12, and 18 1
     • RITAZAREM scheme: 1000 mg infusion after induction of remission, and at months 4,8,12, and 16 1
   • Continue maintenance therapy for 24-48 months, with longer duration for relapsing patients 1

3. Monitoring during rituximab therapy:

   • Measure serum immunoglobulin concentrations prior to each course to detect secondary immunodeficiency 1
   • Use trimethoprim-sulfamethoxazole as prophylaxis against Pneumocystis jirovecii pneumonia 1

For Non-Severe Recurrent Disease:

1. First-line options:

   • Glucocorticoids (prednisolone starting at lower doses of 20-30 mg/day) with steroid-sparing agents 2
   • Steroid-sparing agents to consider:
     • Dapsone (1-2 mg/kg/day) - particularly effective for persistent cutaneous manifestations 3
     • Colchicine - for mild cutaneous and joint manifestations 2
     • Methotrexate - if GFR >60 ml/min per 1.73m² 1

2. For steroid-dependent cases:

   • Mycophenolate mofetil (up to 2g/day) 1, 2
   • Azathioprine (1.5-2 mg/kg/day) 1
   • Consider rituximab to reduce steroid dependency 2

Management of Refractory Disease

For patients who fail to respond to initial therapy:

1. Reassess disease status and comorbidities 1
2. Consider alternative treatments:
   • Switch from cyclophosphamide to rituximab or vice versa 1
   • Consider intravenous immunoglobulin for persistent low activity 1
   • Plasma exchange may be considered in severe cases 1
3. Refer to specialized centers with expertise in vasculitis 1

Important Considerations

• Patients with recurrent IgA vasculitis should be managed in collaboration with centers of expertise in vasculitis 1
• Adult-onset IgA vasculitis tends to be more severe and more likely to recur than childhood-onset disease 4, 5
• Long-term prognosis depends primarily on the severity of renal involvement 5
• Rituximab has demonstrated efficacy in reducing relapse frequency and achieving long-term remission in both children and adults with IgA vasculitis 2

Pitfalls to Avoid

1. Overreliance on glucocorticoids alone - Recent studies show limited effect on long-term outcomes in IgA vasculitis 4
2. Inadequate duration of maintenance therapy - Premature discontinuation increases relapse risk
3. Basing treatment decisions solely on ANCA status - Structured clinical assessment should guide treatment decisions 1
4. Failure to monitor for treatment complications - Regular screening for immunodeficiency, infections, and other adverse effects is essential
5. Neglecting renal monitoring - Renal involvement determines long-term prognosis and requires close monitoring

By following this structured approach and selecting appropriate immunosuppressive therapy based on disease severity, recurrent IgA vasculitis can be effectively managed to prevent organ damage and improve long-term outcomes.