Management of Henoch-Schönlein Purpura (HSP)
The management of Henoch-Schönlein Purpura should focus on supportive care for most patients, with targeted interventions for specific organ involvement and complications, as HSP is typically self-limiting in 94% of children and 89% of adults. 1
General Management Principles
- HSP is an IgA-mediated systemic small-vessel vasculitis affecting the skin, gastrointestinal tract, joints, and kidneys 2
- Most cases resolve spontaneously within approximately 4 weeks, requiring only supportive care 2
- Long-term prognosis depends primarily on the severity of renal involvement 1
Supportive Care
- Provide adequate pain management with acetaminophen as first-line analgesic, avoiding NSAIDs due to potential renal complications 3
- Consider a low-antigen-content (LAC) diet for 4-8 weeks to improve symptoms and laboratory abnormalities in symptomatic patients 3
- Monitor for complications with regular urine testing for proteinuria and hematuria, and blood pressure measurements for at least 6 months 4
Organ-Specific Management
Skin and Joint Manifestations
- For severe cutaneous manifestations and joint pain, oral prednisone at 1-2 mg/kg daily for two weeks may provide symptomatic relief 1, 3
- Colchicine (1 mg/day) can be considered for persistent purpura and pain, with treatment for at least six months 3
- Dapsone may be beneficial for chronic cutaneous manifestations in adults 5
Gastrointestinal Symptoms
- Corticosteroids may be useful for refractory abdominal pain 5
- A meta-analysis found that corticosteroid use in children reduced the mean time to resolution of abdominal pain 1
Renal Involvement
- For HSP nephritis with persistent proteinuria, treatment with ACE inhibitors or ARBs is recommended 3
- 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, a 6-month course of corticosteroid therapy is suggested 3
- For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treatment with steroids and cyclophosphamide is recommended 3
- Prophylactic use of corticosteroids is not recommended to prevent HSP nephritis 3
- In severe cases with renal involvement, consider steroids combined with immunosuppressants such as azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid 3
- End-stage renal disease occurs in 1-5% of patients 1
Special Considerations
Children vs Adults
- HSP affects children more commonly (90% of cases occur in children younger than 10 years) 1
- Adults with HSP are more likely to experience complications and have worse renal prognosis 1, 5
- Treatment approach for HSP nephritis should be the same in adults as in children 3
Pregnancy
- Women with a history of childhood HSP require close monitoring during pregnancy for complications such as proteinuria and hypertension 4
Follow-up Recommendations
- Monitor all patients for at least 6 months 4
- Regular urine testing for proteinuria and hematuria 4
- Regular blood pressure measurements 4
- More intensive monitoring for patients with renal involvement 3
Treatment Algorithm
- Initial presentation: Provide supportive care with acetaminophen for pain
- For severe joint/skin symptoms: Consider prednisone 1-2 mg/kg daily for two weeks
- For persistent GI symptoms: Consider corticosteroids for refractory abdominal pain
- For renal involvement:
- Start ACE inhibitors or ARBs for persistent proteinuria
- Add corticosteroids if proteinuria >1g/day persists despite ACE-I/ARB
- For severe nephritis: Consider combined immunosuppressive therapy
- For all patients: Monitor urine and blood pressure for at least 6 months