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

Treatment of Henoch-Schönlein Purpura (HSP)

Treatment of Henoch-Schönlein purpura (HSP) should be primarily supportive for most cases, with specific interventions tailored to organ involvement severity, particularly renal disease which requires careful monitoring and may need ACE inhibitors/ARBs for persistent proteinuria. 1

General Treatment Approach

• Most cases of HSP are self-limited, with an average duration of 4 weeks 2
• Primary management includes:
  • Adequate hydration
  • Rest during acute phase
  • Regular monitoring of blood pressure and renal function
  • Sodium restriction (enhances antiproteinuric effects in cases with renal involvement) 1

Organ-Specific Management

Skin and Joint Involvement

• Supportive care is usually sufficient
• For recurrent cases with persistent cutaneous and joint symptoms:
  • Dapsone has shown beneficial effects in adults with chronic forms 3
  • Colchicine may be effective for relapsing disease that doesn't respond to ibuprofen or steroids 4

Gastrointestinal Involvement

• Primarily supportive management for mild symptoms
• For severe abdominal pain or gastrointestinal hemorrhage:
  • Oral corticosteroids may be considered 2
  • Monitor for complications such as intussusception 4

Renal Involvement

This requires the most careful monitoring and intervention:

Monitoring Protocol

• Urinalysis with microscopy at diagnosis
• Weekly urinalysis for first month
• Every 2 weeks during second month
• Monthly for next 4-6 months
• Every 3 months until 1 year after diagnosis 1

Treatment Based on Severity

1. No or Mild Renal Involvement:

   • Supportive care and monitoring

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

   • ACE inhibitor or ARB therapy
   • Start with weight-appropriate dose
   • Gradually increase to maximum tolerated dose over 2-4 weeks
   • Monitor serum creatinine and potassium 1-2 weeks after initiation and with each dose increase 1

3. Heavy Proteinuria (>1 g/day/1.73 m²) Despite 3-6 Months of ACE/ARB:

   • Consider adding corticosteroids 1
   • Consider renal biopsy

4. Severe Nephritis or Deteriorating Kidney Function:

   • High-dose intravenous corticosteroids plus cyclophosphamide 1
   • Alternative immunosuppressants (cyclosporine or mycophenolate mofetil) when cyclophosphamide is contraindicated

Important Caveats

• Corticosteroids should NOT be used prophylactically to prevent HSP nephritis 1
• Early steroid treatment does not reduce the incidence or severity of nephropathy in children with HSP 2
• Renal involvement is the most important prognostic factor determining long-term morbidity and mortality 2
• Adults with HSP should be treated similarly to children, but they often have more severe initial renal involvement and worse prognosis 1
• The evidence for cyclophosphamide in severe HSP-associated kidney disease is limited, with studies showing no significant benefit over supportive care 5

Long-term Management

• Continue ACE inhibitor/ARB therapy for at least 12 months after achieving remission
• Gradually taper if proteinuria resolves completely
• Monitor for relapse after medication discontinuation
• Consider lipid-lowering therapy if persistent hyperlipidemia is present 1

For patients with severe nephritis or persistent proteinuria >3 months, an ACE inhibitor or ARB should be considered in addition to corticosteroids to prevent secondary glomerular injury 2.