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

Treatment of IgA Vasculitis

For IgA vasculitis with severe kidney disease (proteinuria, declining renal function, or active glomerulonephritis), initiate prednisone as first-line therapy; for isolated cutaneous or mild disease without organ involvement, supportive care alone is often sufficient, though glucocorticoids can be used for symptom control. 1

Disease Severity Stratification

The treatment approach fundamentally depends on the presence and severity of organ involvement, particularly renal disease:

• Severe kidney disease is defined by significant proteinuria, declining glomerular filtration rate, active glomerulonephritis on biopsy, or crescentic changes 1
• Mild disease includes isolated purpura, arthralgia, or mild gastrointestinal symptoms without organ-threatening features 2, 3
• Adults typically present with more severe disease than children, with 10-30% progressing to end-stage renal disease compared to much lower rates in pediatric populations 2, 3

Treatment Algorithm by Severity

Severe Kidney Disease or Organ-Threatening Manifestations

Prednisone is the recommended first-line therapy for patients with IgA vasculitis and severe kidney disease 1. The KDIGO 2021 guidelines specifically address this population, though the evidence base remains limited.

• Initiate prednisone at 0.5-1 mg/kg/day (maximum 60-80 mg daily) 2
• Taper glucocorticoids to a maintenance dose of ≤10 mg/day prednisolone during remission 1
• Continue tapering gradually after 6-18 months depending on response, with the goal of discontinuation 1

For severe gastrointestinal manifestations (hemorrhage, intussusception, perforation risk), high-dose intravenous corticosteroids are indicated 4, 2. One case series demonstrated resolution of severe GI symptoms including hematemesis and hematochezia with IV corticosteroids combined with proton pump inhibitors 4.

Glucocorticoid-Sparing and Adjunctive Immunosuppression

When glucocorticoids alone are insufficient, or to reduce cumulative steroid burden in relapsing disease:

• Mycophenolate mofetil (2000 mg/day in divided doses) has shown favorable results as a glucocorticoid-sparing agent 2
• Calcineurin inhibitors (cyclosporine A or tacrolimus) can be effective for refractory cases 2
• Rituximab (375 mg/m² weekly for 4 weeks) has demonstrated efficacy in reducing relapse frequency and achieving long-term remission in both children and adults with severe or relapsing IgAV 2
• Azathioprine (1.5-2 mg/kg/day) or methotrexate (15-25 mg/week) can be used for maintenance therapy 5, 2

Mild or Self-Limited Disease

For isolated cutaneous manifestations, arthralgia, or mild gastrointestinal symptoms without organ involvement:

• Supportive care alone is often sufficient, as the disease is typically self-limited in these cases 3
• Symptomatic treatment with NSAIDs for arthralgia (use cautiously given potential renal involvement) 3
• Colchicine or dapsone may be useful for controlling minor cutaneous manifestations 2
• Short courses of low-dose glucocorticoids can be considered for symptom control if needed 2, 3

Special Considerations and Pitfalls

Steroid-Dependent or Relapsing Disease

A critical pitfall is the development of steroid dependence with recurrent flares upon tapering 6. Recent evidence suggests corticosteroids have limited effect on long-term outcomes, particularly renal progression 6.

• For steroid-dependent disease, transition to steroid-sparing agents rather than prolonging high-dose corticosteroids 6
• Rituximab is particularly effective for reducing cumulative glucocorticoid burden and achieving sustained remission 2
• Monitor for steroid-induced complications including hyperglycemia, especially during prolonged therapy 6

Gastrointestinal Infections

Patients with IgAV are at increased risk for severe gastrointestinal infections that can complicate the disease course 4. In a case-based review, 42.9% had bacterial pathogens, 25.7% viral (including norovirus), and 17.1% parasitic infections 4.

• Maintain high clinical suspicion for superimposed GI infections in patients with severe abdominal symptoms 4
• Obtain stool studies when appropriate before attributing all GI symptoms to vasculitis activity 4
• Severe complications from infections can cause significant morbidity, particularly in adults 4

Adult vs. Pediatric Considerations

Adults with IgAV require more aggressive monitoring and often more intensive treatment 2, 3:

• Higher frequency of glomerulonephritis (up to 50-80% in adults vs. 20-40% in children) 2
• Greater risk of progression to chronic kidney disease and end-stage renal disease 2, 3
• More severe initial presentation and worse long-term prognosis 3, 7
• Lower threshold for initiating immunosuppressive therapy in adults with any renal involvement 2

Monitoring Requirements

• Urinalysis and renal function should be monitored regularly, as long-term prognosis depends primarily on the severity of nephritis 3
• Renal biopsy should be considered for significant proteinuria or declining renal function to guide treatment intensity 1
• Monitor for disease relapse, which occurs in approximately 30-40% of cases, typically within the first year 2

Emerging and Investigational Therapies

While not yet standard of care, several therapies show promise for refractory cases 2:

• Intravenous immunoglobulin (IVIG) for severe or life-threatening situations
• Plasma exchange for rapidly progressive glomerulonephritis
• Leflunomide (requires further study)
• TRF-budesonide, SGLT2 inhibitors, and complement pathway inhibitors (investigational)

The key principle is that treatment intensity should match disease severity, with supportive care for mild self-limited disease and aggressive immunosuppression reserved for organ-threatening manifestations, particularly severe renal involvement. 1, 2, 3