What is the treatment for Henoch-Schönlein purpura (HSP)?

Treatment of Henoch-Schönlein Purpura (HSP)

The treatment of Henoch-Schönlein purpura should be tailored to disease severity, with most cases requiring only supportive care, while ACE inhibitors or ARBs should be used as first-line therapy for persistent proteinuria, and immunosuppressive therapy reserved for severe cases with crescentic nephritis or deteriorating kidney function. 1, 2

General Management Approach

Mild HSP (No Significant Organ Involvement)

• Most cases of HSP are self-limited and resolve spontaneously within 4-6 weeks 3
• Treatment is primarily supportive:
  • NSAIDs for joint pain and mild abdominal discomfort 4
  • Adequate hydration
  • Rest during acute phase
• Corticosteroids should NOT be used to prevent HSP nephritis (strong evidence against this practice) 1

Monitoring

• Regular urinalysis to detect kidney involvement
• Blood pressure monitoring
• Renal function tests if proteinuria or hematuria develops

Treatment of HSP with Kidney Involvement

Persistent Proteinuria (0.5-1 g/day/1.73m²)

• First-line therapy: ACE inhibitors or ARBs 1, 2
  • Start with weight-appropriate dosing
  • Titrate to maximum tolerated dose over 2-4 weeks
  • Monitor serum creatinine and potassium 1-2 weeks after initiation and with each dose increase
  • Target blood pressure: 50th percentile for age, sex, and height
  • Hold medication if serum creatinine increases >30% from baseline

Persistent Proteinuria >1 g/day/1.73m² Despite ACE-I/ARB

• Add corticosteroids if GFR >50 ml/min/1.73m² and proteinuria persists after 3-6 months of optimized ACE-I/ARB therapy 1, 2
• Corticosteroid therapy should be considered only if nephrotic-range proteinuria has not improved after a trial of angiotensin blockade 1

Severe HSP Nephritis (Crescentic HSP with Nephrotic Syndrome or Deteriorating Kidney Function)

• High-dose intravenous corticosteroid therapy plus cyclophosphamide (treated the same as crescentic IgA nephropathy) 1
• Alternative immunosuppressants when cyclophosphamide is contraindicated:
  • Cyclosporine may be effective for heavy proteinuria and crescentic HSP, though evidence for long-term outcomes is limited 1
  • Mycophenolate mofetil has shown effectiveness in some cases 5

Treatment Based on Specific Manifestations

Gastrointestinal Involvement

• Severe or persistent GI symptoms may require:
  • Corticosteroids for significant abdominal pain
  • Mycophenolate mofetil may be effective for steroid-resistant cases 5
  • Monitor for intussusception, which requires urgent surgical evaluation

Recurrent HSP

• Colchicine may be effective in patients with relapsing disease that doesn't respond to NSAIDs or steroids 6

Important Caveats and Pitfalls

1. Do not use corticosteroids prophylactically - There is moderate quality evidence that corticosteroids are not beneficial at the onset of HSP to prevent nephritis 1, 7

2. Renal biopsy indications:

   • Decreased renal function at presentation
   • Severe nephrotic/nephritic syndrome
   • Persistent heavy proteinuria despite optimal therapy 1

3. Adult vs. Pediatric Treatment:

   • Adults with HSP should be treated the same as children, though adults may have more severe initial renal involvement 1
   • Prognosis in adult HSP is generally worse with protein excretion >1 g/day 1

4. Medication Precautions:

   • Counsel patients/parents to temporarily stop ACE-I/ARB during illness with risk of dehydration 2
   • Monitor for nephrotoxicity with cyclosporine use, especially in children with relapse after initial therapy 1

The evidence for treatment of HSP nephritis is limited, with most recommendations based on extrapolation from IgA nephropathy studies. Current guidelines emphasize the importance of targeting therapy to disease severity rather than treating all patients with immunosuppression.