Management of Henoch-Schönlein Purpura (HSP)
For patients with Henoch-Schönlein purpura, treatment should focus on supportive care for mild cases, with specific interventions for organ involvement, particularly using ACE inhibitors or ARBs for nephritis with proteinuria, and corticosteroids for severe manifestations.
Clinical Presentation and Diagnosis
HSP is an acute, systemic, immune complex-mediated leukocytoclastic vasculitis characterized by:
- Palpable purpura (without thrombocytopenia)
- Abdominal pain
- Arthritis
- Potential renal involvement
The condition predominantly affects children under 10 years (>90% of cases), but adults may experience more severe complications 1.
Key Clinical Manifestations:
- Skin: Palpable purpura (100% of patients), predominantly on lower extremities
- Joints: Arthritis (75% of patients)
- Gastrointestinal: Abdominal pain (60-65% of patients)
- Renal: Hematuria, proteinuria (40-50% of patients)
Management Approach
1. General Supportive Care
Most cases resolve spontaneously (94% in children, 89% in adults) 1, making supportive care the primary intervention:
- Adequate hydration
- Pain management for joint symptoms
- Monitoring for complications
2. Renal Involvement Management
For children with HSP nephritis and persistent proteinuria (0.5-1 g/day per 1.73 m²):
- Treat with ACE inhibitors or ARBs 2
For children with persistent proteinuria >1 g/day per 1.73 m² after ACE inhibitor/ARB trial and GFR >50 ml/min per 1.73 m²:
- Implement a 6-month course of corticosteroid therapy 2
For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function:
- Treat as for crescentic IgA nephropathy with steroids and cyclophosphamide 2
3. Gastrointestinal and Joint Symptoms
For significant abdominal pain and joint symptoms:
- Oral prednisone at 1-2 mg/kg daily for two weeks 1
- A meta-analysis showed that corticosteroid use in children reduced the mean time to resolution of abdominal pain 1
4. Severe or Refractory Cases
For life-threatening manifestations or compromised renal function:
- Methylprednisolone pulse therapy
- Immunosuppressive drugs (cyclophosphamide, azathioprine)
- Plasma exchange in select cases
- Polyclonal immunoglobulin therapy 3
In adults with chronic forms, dapsone may have beneficial effects on cutaneous, gastrointestinal, and articular manifestations 3.
Special Considerations
Adult vs. Pediatric Management
- Adult HSP patients should be treated similarly to children 2
- However, adults generally have worse renal prognosis than children 3
Monitoring and Follow-up
Patients should be monitored for:
- Resolution of skin manifestations
- Improvement in abdominal and joint symptoms
- Renal function and proteinuria
- Potential complications
Prognosis
Long-term prognosis depends primarily on the severity of renal involvement:
- End-stage renal disease occurs in 1-5% of patients 1
- Adults have worse renal outcomes compared to children 3
Cautions and Contraindications
- Corticosteroids: Not recommended for prevention of HSP nephritis (strong evidence, 1B) 2
- Immunosuppressants: Should be used cautiously in patients with infections
- Monitoring: Regular assessment of renal function is essential in all HSP patients
Patient Education
Patients and caregivers should be informed about:
- The typically self-limiting nature of the disease
- Warning signs requiring medical attention (severe abdominal pain, significant hematuria)
- Importance of follow-up, especially for monitoring renal function
- Expected timeline for symptom resolution
A multidisciplinary team approach with clear communication between healthcare providers is essential for optimal management of HSP patients 2.