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

Treatment of IgA Vasculitis in Adults

For adults with IgA vasculitis (IgAV), treatment should be guided by disease severity, with glucocorticoids as first-line therapy for severe manifestations, while supportive care alone is appropriate for self-limited disease without organ involvement. 1

Disease Classification and Initial Assessment

IgA vasculitis in adults differs significantly from the pediatric form, with higher risk of progression to end-stage renal disease and generally more severe manifestations. When evaluating an adult with IgAV, categorize the disease based on severity:

• Non-severe disease: Isolated cutaneous manifestations, mild arthralgia/arthritis
• Severe disease: Significant organ involvement including:
  • Renal involvement (IgAV nephritis)
  • Gastrointestinal complications
  • Pulmonary or cardiac manifestations
  • Neurological involvement

Adults with IgAV should be assessed for secondary causes and undergo age- and sex-appropriate malignancy screening 1.

Treatment Algorithm

1. Non-severe IgAV (Isolated cutaneous/mild joint involvement)

• First-line: Supportive care only 1, 2

  • Adequate hydration
  • Pain management with acetaminophen or NSAIDs if no renal involvement
  • Rest and elevation of affected limbs for purpura

• For persistent cutaneous or mild joint symptoms:

  • Consider colchicine, dapsone, or methotrexate 3

2. Severe IgAV with Renal Involvement (IgAV Nephritis)

Renal involvement determines long-term prognosis in adults with IgAV 2.

• For proteinuria without nephrotic syndrome:

  • ACE inhibitors or ARBs for blood pressure control and proteinuria reduction 1
  • Monitor renal function and proteinuria closely

• For nephrotic syndrome, rapidly deteriorating kidney function, or crescentic glomerulonephritis:

  • High-dose glucocorticoids (oral prednisone/prednisolone or IV methylprednisolone) 1, 3
  • Consider adding immunosuppressive therapy:
    • Cyclophosphamide (although evidence shows it may not improve renal outcomes compared to supportive care alone) 4, 5
    • Mycophenolate mofetil as an alternative to cyclophosphamide 3, 5
    • Rituximab has shown promise in reducing relapse frequency and achieving long-term remission 3, 4

3. Severe IgAV with Significant Gastrointestinal Involvement

• For severe abdominal pain, GI bleeding:
  • High-dose glucocorticoids 1, 2
  • For life-threatening complications:
    • Consider plasma exchange in addition to glucocorticoid therapy 1
    • IV immunoglobulins may be beneficial in difficult cases 3

4. Rapidly Progressive IgAV with Multiple Organ Involvement

• For rapidly progressive disease with multiple organ involvement:
  • High-dose glucocorticoids plus cyclophosphamide or rituximab 1, 3
  • Treatment approach similar to ANCA-associated vasculitis protocols 1

Monitoring and Follow-up

• Regular monitoring of renal function, urinalysis, and proteinuria
• Follow-up duration should be at least 6-12 months, even after resolution of symptoms
• Adults with history of IgAV nephritis require long-term monitoring for chronic kidney disease

Important Considerations and Pitfalls

1. Adult vs. Pediatric IgAV: Unlike in children, IgAV in adults carries a worse prognosis with higher risk of progression to end-stage renal disease (10-30% of adults) 3.

2. Evidence Limitations: High-quality evidence for treatment of IgAV in adults is lacking. Most recommendations are extrapolated from pediatric studies or small case series 6, 5.

3. Glucocorticoid Caution: While glucocorticoids are first-line for severe manifestations, they should not be used prophylactically to prevent nephritis in patients with isolated extrarenal IgAV (Grade 1B recommendation) 1.

4. Renal Biopsy: Consider renal biopsy in adults with significant proteinuria, hematuria, or declining renal function to guide treatment decisions.

5. Malignancy Association: Adult-onset IgAV may be associated with underlying malignancy, particularly in older adults, necessitating appropriate screening 1.

The treatment of IgAV in adults remains challenging due to limited high-quality evidence. While glucocorticoids form the backbone of therapy for severe disease, the optimal immunosuppressive regimen remains to be defined through controlled trials.