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

Treatment of IgA Vasculitis

For IgA vasculitis (Henoch-Schönlein purpura), glucocorticoids are the first-line therapy for severe manifestations, particularly in adults, while self-limited disease requires only symptomatic treatment. 1

Disease Severity Assessment and Initial Management

The treatment approach depends critically on disease severity and organ involvement:

• Self-limited disease (isolated purpura, mild arthralgia without organ involvement) requires only symptomatic treatment with rest, hydration, and analgesics 2, 3
• Severe disease is defined by significant renal involvement (proteinuria >1g/day, declining GFR, active urinary sediment), severe gastrointestinal manifestations (hematemesis, hematochezia, intussusception), or other organ-threatening features 1, 3

Critical Initial Assessment

Before initiating treatment, evaluate for:

• Renal involvement: Urinalysis for hematuria, proteinuria, red cell casts; serum creatinine and GFR calculation 3
• Gastrointestinal severity: Assess for hematemesis, hematochezia, severe abdominal pain suggesting bowel ischemia or intussusception 4, 3
• Exclude concurrent infections: Stool studies if severe GI symptoms present, as bacterial (42.9%), viral (25.7%), or parasitic (17.1%) infections can complicate IgAV and require specific management 4

Treatment Algorithm by Disease Severity

Mild to Moderate Disease (No Organ-Threatening Features)

For isolated cutaneous manifestations or mild arthritis:

• Symptomatic management with NSAIDs for pain control and supportive care 2, 3
• Colchicine, dapsone, or methotrexate can be useful for controlling persistent minor manifestations 1
• Monitor closely for development of renal or severe GI involvement with serial urinalysis and clinical assessment 3

Severe Disease (Organ-Threatening Manifestations)

Glucocorticoids are the cornerstone of therapy:

• Prednisone 1 mg/kg/day (maximum 60-80 mg/day) for induction of remission 1, 3
• Continue high-dose therapy for 2-4 weeks, then taper gradually over 2-3 months based on clinical response 1
• Proton pump inhibitors should be added for severe gastrointestinal manifestations 4

Severe Renal Involvement (IgA Vasculitis Nephritis)

For patients with significant proteinuria, declining renal function, or crescentic glomerulonephritis:

• Glucocorticoids combined with immunosuppressive agents are recommended, though evidence is limited 2, 1, 3
• Immunomodulatory options include:
  • Cyclosporine A, tacrolimus, or mycophenolate mofetil as glucocorticoid-sparing agents with favorable results 1
  • Cyclophosphamide may be considered for rapidly progressive glomerulonephritis, though data are extrapolated from ANCA vasculitis experience 1

Refractory or Relapsing Disease

Rituximab for Resistant Cases

Rituximab has emerged as the most effective therapy for glucocorticoid-resistant or refractory IgA vasculitis:

• 94.3% of patients achieve clinical improvement and 74.3% achieve sustained remission with rituximab 5
• Indicated for patients resistant or refractory to glucocorticoids or other immunosuppressive drugs (85.7% of treated cases) 5
• Standard dosing: 375 mg/m² weekly for 4 weeks, or 1000 mg on days 1 and 15 5
• Relapse management: Among patients who relapse (37.1%), repeat rituximab dosing achieves good disease control in all cases 5
• Significantly reduces glucocorticoid burden and additional immunosuppressant requirements 5
• Safety profile is favorable with only 8.6% minor adverse effects and no deaths reported 5

Alternative Therapies for Difficult Cases

• Intravenous immunoglobulin (IVIG) and plasma exchange can be useful in life-threatening situations 1
• Leflunomide shows promising results but requires further study 1

Special Considerations and Pitfalls

Adult vs. Pediatric Disease

• Adults have worse prognosis with more frequent and severe nephritis (10-30% progress to end-stage renal disease vs. lower rates in children) 2, 1, 3
• IgA vasculitis is 150-200 times less common in adults than children, with annual incidence of 1 in 1 million 2, 3
• One-third of adult patients may reach end-stage renal failure with prolonged follow-up 2

Infection-Related Complications

Common pitfall: Failing to recognize concurrent gastrointestinal infections that can mimic or exacerbate IgAV:

• Bacterial pathogens account for 42.9% of GI infections complicating IgAV 4
• Norovirus and other viral gastroenteritis (25.7%) can cause severe symptoms requiring differentiation from primary vasculitis activity 4
• Obtain stool studies before escalating immunosuppression in patients with severe GI symptoms 4

Monitoring and Long-Term Management

• Short-term outcome depends on severity of gastrointestinal manifestations 2, 3
• Long-term prognosis is heavily dependent on presence and severity of nephritis 2, 3
• Serial monitoring of urinalysis and renal function is essential, as renal involvement can develop or progress even after initial presentation 3
• Some authors suggest IgA nephropathy and IgA vasculitis represent the same disease spectrum 2

Treatment Duration

• Maintenance therapy should continue for at least 18-24 months in patients with severe disease who achieve remission 1
• Glucocorticoid taper should be gradual to minimize relapse risk 1
• Consider rituximab for maintenance in relapsing disease to reduce long-term glucocorticoid exposure 5