What are the diagnosis and treatment options for Henoch-Schonlein Purpura (HSP)?

Diagnosis and Treatment of Henoch-Schönlein Purpura (HSP)

Henoch-Schönlein purpura is diagnosed through clinical presentation of palpable purpura plus at least one of the following: abdominal pain, IgA deposition, arthritis/arthralgia, or renal involvement, with treatment focused on supportive care for mild cases and immunosuppressive therapy for organ involvement.

Diagnosis

HSP is a systemic vasculitis primarily affecting small and medium-sized blood vessels. The diagnosis is primarily clinical, based on characteristic findings:

Key Diagnostic Features:

• Mandatory feature: Palpable purpura (100% of cases) 1
• Plus at least one of the following:
  • Abdominal pain (56% of cases) 2
  • IgA deposition on tissue biopsy
  • Arthritis or arthralgia (66% of cases) 2
  • Renal involvement (30% of cases) 2

Diagnostic Workup:

1. Complete blood count: To assess for inflammation and rule out other causes
2. Urinalysis: To detect hematuria and/or proteinuria indicating renal involvement
3. Renal function tests: BUN, creatinine to assess kidney function
4. Skin biopsy: Shows leukocytoclastic vasculitis with IgA deposition in vessel walls 1
5. Abdominal imaging: Ultrasound is recommended as first-line test if GI symptoms are present to rule out intussusception 3

Treatment

Treatment approach depends on disease severity and organ involvement:

Mild Disease (Skin and Joint Involvement Only):

• Supportive care
• NSAIDs for joint pain
• Regular monitoring for development of renal involvement

Gastrointestinal Involvement:

• Corticosteroids: For severe abdominal pain or GI bleeding 2
  • Helps prevent complications such as GI bleeding and intussusception
  • Dosage: Prednisone 1-2 mg/kg/day (based on clinical practice)

Renal Involvement:

Treatment escalation based on severity:

1. Mild nephritis (microscopic hematuria, minimal proteinuria):

   • Close monitoring
   • Some cases resolve spontaneously 2

2. Moderate-to-severe nephritis (nephrotic-range proteinuria):

   • Corticosteroids: Prednisone 1-2 mg/kg/day 2
   • For non-responders: Add cyclophosphamide 2

3. Severe/resistant nephritis:

   • Combined therapy with steroids and cyclophosphamide 2
   • Alternative options for steroid/cyclophosphamide-resistant cases:
     • Cyclosporine A 2
     • Mycophenolate mofetil 1
     • Rituximab (promising but requires more studies) 1

Severe Multi-System Disease:

• Pulse steroid therapy (methylprednisolone 10-30 mg/kg/day for 3 days) 4
• Consider cyclophosphamide for severe multi-organ involvement 4

Follow-up and Monitoring

• All patients should be monitored for at least 6 months after diagnosis 5
• Regular urinalysis to detect proteinuria and hematuria 5
• Regular blood pressure measurements 5
• More intensive monitoring for those with renal involvement

Special Populations

Children:

• Most common systemic vasculitis in childhood 3
• Generally better prognosis than adults
• Focus on preventing long-term renal complications

Adults/Elderly:

• Rare presentation but typically more severe 4
• Higher risk of severe complications and rapid progression 4
• Consider cancer screening in older males with HSP 1

Pregnant Women:

• Women with history of childhood HSP need close monitoring during pregnancy
• Increased risk of proteinuria and hypertension during pregnancy 5

Complications to Monitor

1. Gastrointestinal:

   • Intussusception (most common surgical complication) 3
   • GI bleeding
   • Bowel perforation (usually ileal) 3

2. Renal:

   • Progressive nephritis
   • Long-term renal insufficiency

3. Other:

   • Neurological manifestations (rare) 4
   • Extensive skin vasculitis 4

The prognosis of HSP is generally good, with most cases self-resolving within 4-6 weeks. However, renal involvement is the most significant predictor of long-term morbidity and requires close monitoring and aggressive treatment when indicated 5.