What is the treatment approach for Henloch-Schonlein purpura (HSP)?

Treatment Approach for Henoch-Schönlein Purpura (HSP)

Most HSP cases are self-limited and require only supportive care with monitoring for renal complications, which are the primary determinant of long-term outcomes. 1, 2

Initial Management Strategy

Supportive Care for Uncomplicated HSP

• Provide symptomatic treatment with acetaminophen for pain control rather than NSAIDs like ketorolac (Toradol), which can cause acute kidney injury in patients with potential renal involvement. 1
• Consider oral prednisone (1-2 mg/kg daily for two weeks) for severe joint pain, widespread cutaneous symptoms, or refractory abdominal pain. 1, 3
• Do NOT routinely use corticosteroids at HSP presentation to prevent nephritis—this is a strong recommendation based on moderate quality evidence showing no benefit. 4, 5

Mandatory Renal Monitoring Protocol

• Perform urinalysis with microscopy at presentation and on day 7 to assess for hematuria, proteinuria, red blood cell casts, and dysmorphic red blood cells. 1
• A normal urinalysis on day 7 has a 97% negative predictive value for normal renal outcome. 6
• Monitor all HSP patients for 6 months with regular urinalysis and blood pressure checks, as renal involvement can develop after initial presentation. 1, 6
• Obtain basic metabolic panel (BUN, creatinine) and complete blood count at baseline. 1

Management of HSP Nephritis

Mild Renal Involvement (Persistent Proteinuria 0.5-1 g/day/1.73 m²)

• Initiate ACE inhibitor or ARB therapy for children with persistent proteinuria despite supportive care. 4, 1
• This recommendation is based on extrapolation from IgA nephropathy data, as direct evidence in HSP is limited. 4

Moderate Renal Involvement (Proteinuria >1 g/day/1.73 m²)

• First, trial ACE inhibitor or ARB therapy. 4, 1
• If proteinuria persists after ACE inhibitor/ARB trial AND GFR >50 ml/min/1.73 m², initiate a 6-month course of corticosteroid therapy (same regimen as for IgA nephropathy). 4, 1
• The threshold for corticosteroid treatment should be nephrotic-range proteinuria that has not improved with angiotensin blockade, not simply proteinuria >1 g/day. 4

Severe/Crescentic HSP Nephritis

• Define crescentic HSP as >50% of glomeruli with crescents on biopsy plus rapidly progressive renal deterioration. 4
• Treat with high-dose corticosteroids (methylprednisolone pulse therapy) combined with cyclophosphamide, analogous to ANCA vasculitis treatment. 4, 1, 3
• This applies to patients with nephrotic syndrome and/or deteriorating kidney function. 4
• Alternative immunosuppressive agents include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil, though evidence is limited. 1, 5

Special Considerations

When to Perform Renal Biopsy

• Perform renal biopsy in children with decreased renal function at presentation or severe nephrotic/nephritic syndrome. 4
• Consider biopsy for persistent nephrotic-range proteinuria to guide aggressive immunosuppression decisions. 1

Adult HSP Management

• Treat adult HSP nephritis identically to children, though adults tend to have worse renal prognosis and more severe initial involvement. 4, 3

Therapies NOT Recommended

• Do NOT use prophylactic corticosteroids to prevent HSP nephritis (strong evidence against this practice). 4, 5
• Do NOT use antiplatelet agents—no evidence of benefit in preventing persistent kidney disease. 4, 5
• Cyclophosphamide showed no significant benefit in two studies for severe HSP nephritis, though it remains recommended for crescentic disease based on extrapolation from other vasculitides. 5

Adjunctive Therapies

• Dapsone may benefit chronic cutaneous, gastrointestinal, and articular manifestations in adults. 3
• Colchicine (1 mg/day for ≥6 months) may be considered for persistent purpura and pain. 1
• A low-antigen-content diet for 4-8 weeks may improve symptoms when strictly followed. 1

Critical Pitfall to Avoid

The most important pitfall is failing to monitor for renal involvement, as approximately 46% of children develop initial renal inflammation, and 2% progress to renal failure. 6, 2 Older children are at higher risk for requiring renal referral. 6 The 6-month monitoring period is essential because renal disease can develop after initial presentation. 1, 6