What is the management of Henoch-Schönlein Purpura (HSP)?

From the Guidelines

The management of Henoch-Schönlein purpura (HSP) primarily focuses on supportive care and symptom management, as most cases resolve spontaneously within 4-6 weeks. For mild cases with primarily skin manifestations, rest, adequate hydration, and acetaminophen (10-15 mg/kg/dose every 4-6 hours, maximum 75 mg/kg/day or 4000 mg/day in adults) are recommended for pain relief. NSAIDs like ibuprofen (10 mg/kg/dose every 6-8 hours, maximum 40 mg/kg/day or 3200 mg/day in adults) can help with joint pain but should be avoided if renal involvement is present. For moderate to severe cases, especially those with significant abdominal pain, arthritis, or renal involvement, corticosteroids such as prednisone (1-2 mg/kg/day, maximum 60-80 mg/day for 1-2 weeks with gradual tapering) may be prescribed, as suggested by 1. Severe cases with significant renal involvement might require immunosuppressive therapy like cyclophosphamide, azathioprine, or mycophenolate mofetil under specialist supervision, with a preference for combining steroid therapy with one of azathioprine, cyclosporine, tacrolimus, or MMF/mycophenolic acid, as noted in 1. Regular monitoring of blood pressure, urinalysis, and renal function is essential, particularly in the first 6 months after diagnosis when renal complications are most likely to develop. In cases with persistent significant proteinuria, angiotensin blockade with ACE inhibitors or ARBs may be considered, as recommended by 1 and supported by 1. Key considerations in the management of HSP include:

• Symptom management with rest, hydration, and pain relief
• Avoidance of NSAIDs in cases with renal involvement
• Use of corticosteroids for moderate to severe cases
• Consideration of immunosuppressive therapy for severe cases with renal involvement
• Regular monitoring of renal function and blood pressure
• Use of angiotensin blockade for cases with persistent significant proteinuria, as suggested by the most recent and highest quality study 1.

From the Research

Management of Henoch-Schönlein Purpura (HSP)

The management of HSP can be divided into several categories, including:

• Supportive care: Most cases of HSP are self-limited and require only supportive care 2
• Corticosteroid treatment: Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat abdominal and joint symptoms 3
• Immunosuppressants: Early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement 3, 4
• Plasma exchange: The addition of plasma exchange to glucocorticosteroids has been shown to be effective in the treatment of severe HSP in adults 5
• Rituximab: Rituximab has been reported as beneficial for children with substantial renal or central nervous system involvement, and has been shown to reduce the number of hospital admissions and oral CS burden in children with chronic CS dependent HSP 6

Treatment of Specific Symptoms

• Abdominal pain: Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat abdominal pain 3
• Joint symptoms: Oral prednisone at 1 to 2 mg per kg daily for two weeks has been used to treat joint symptoms 3
• Renal involvement: Early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement 3, 4
• Nephritis: An angiotensin-converting enzyme inhibitor or angiotensin receptor blocker should be considered in addition to corticosteroids to prevent and/or limit secondary glomerular injury in patients with severe nephritis or renal involvement with proteinuria of greater than 3 months 2

Special Considerations

• Children: Most cases of HSP in children are self-limited and require only supportive care, but corticosteroid treatment may be considered for severe gastrointestinal pain and gastrointestinal hemorrhage 2
• Adults: Adult HSP has been associated with poor outcome and end-stage renal disease in >20% of cases, and the addition of plasma exchange to glucocorticosteroids has been shown to be effective in the treatment of severe HSP in adults 5