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

Treatment of Henoch-Schönlein Purpura (HSP)

The treatment of Henoch-Schönlein Purpura (HSP) should be primarily supportive for most patients, with specific interventions targeted at organ involvement, particularly for renal manifestations which determine long-term prognosis.

General Management Principles

• HSP is typically self-limiting, with spontaneous resolution occurring in 94% of children and 89% of adults, making supportive care the primary intervention 1
• Provide patient information and treat pain if needed, using acetaminophen as a first-line analgesic option rather than NSAIDs due to potential renal complications 2
• Monitor all patients with HSP for at least 6 months with regular urine testing for proteinuria and hematuria, and blood pressure measurements 3

Treatment Based on Organ System Involvement

Skin and Joint Involvement

• For joint pain and cutaneous manifestations, supportive care is generally sufficient 1
• For severe joint pain, oral prednisone at 1-2 mg/kg daily for two weeks may be considered 1

Gastrointestinal Involvement

• For severe gastrointestinal pain or gastrointestinal hemorrhage, oral corticosteroids may be considered 4
• A meta-analysis found that corticosteroid use in children reduced the mean time to resolution of abdominal pain 1

Renal Involvement

• For persistent proteinuria >1 g/day per 1.73 m², treatment with ACE inhibitors or ARBs is recommended 2
• If proteinuria persists despite ACE inhibitor/ARB therapy and GFR >50 ml/min per 1.73 m², a 6-month course of corticosteroid therapy should be considered 2
• For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treatment with steroids and cyclophosphamide is recommended 2, 5
• For severe nephritis requiring additional therapy, options include steroids combined with azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil 2
• Prophylactic use of corticosteroids is not recommended to prevent HSP nephritis 2

Special Considerations

Adults vs. Children

• Adults with HSP should be treated with the same approach as children 2, 5
• However, adults are more likely to experience complications than children and may have worse renal prognosis 1, 6

Severe Disease

• For rare life-threatening forms or cases with compromised renal function, consider methylprednisolone pulse therapy, immunosuppressive drugs (cyclophosphamide or azathioprine), plasma exchange, or polyclonal immunoglobulin therapy 6
• Early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement 1

Long-term Prognosis

• Long-term prognosis depends primarily on the severity of renal involvement 1, 4
• End-stage renal disease occurs in 1-5% of patients 1
• Women with a history of childhood HSP require close monitoring during pregnancy for complications such as proteinuria and hypertension 3

Treatment Algorithm

1. Initial presentation: Supportive care for all patients
2. Pain management: Acetaminophen as first-line; consider prednisone for severe joint or abdominal pain
3. Renal monitoring: Regular urine testing and blood pressure measurement for at least 6 months
4. For persistent proteinuria: Start ACE inhibitor or ARB
5. If proteinuria persists despite ACE/ARB: Add 6-month course of corticosteroids
6. For severe nephritis or deteriorating renal function: Combination of steroids with immunosuppressants (cyclophosphamide preferred for crescentic nephritis)