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

Treatment of Henoch-Schönlein Purpura

Most HSP cases are self-limited and require only supportive care, with treatment reserved for specific complications—particularly renal involvement, severe gastrointestinal symptoms, or joint pain. 1, 2

Initial Management and Supportive Care

• Provide symptomatic relief with acetaminophen for pain and fever—avoid NSAIDs like ketorolac (Toradol) as they can cause acute kidney injury, especially problematic given the risk of renal involvement in HSP 1
• Apply appropriate wound care with dressings for any skin lesions that may weep or become secondarily infected 1
• Monitor blood pressure at every visit, as hypertension indicates more severe renal involvement 1

Renal Disease Management (The Most Critical Aspect)

For Mild Renal Involvement (Microscopic Hematuria, Proteinuria <1 g/day/1.73 m²)

• Start ACE inhibitor or ARB therapy for persistent significant proteinuria, even though evidence is extrapolated from IgA nephropathy rather than HSP-specific trials 3, 1
• Target proteinuria to <1 g/day/1.73 m² rather than attempting complete normalization, which increases medication side effects without proven benefit 3
• Continue ACE inhibitor/ARB for at least 90 days before considering any immunosuppressive therapy 1

For Moderate Renal Involvement (Persistent Proteinuria >1 g/day/1.73 m² After ACE Inhibitor/ARB Trial)

• Initiate a 6-month course of oral corticosteroids only if nephrotic-range proteinuria persists after adequate trial of angiotensin blockade 3, 1
• The KDOQI commentary explicitly disagrees with using corticosteroids for proteinuria thresholds of 1 g/day/1.73 m², stating limited data support steroid therapy at this level 3
• Reserve corticosteroid therapy for nephrotic syndrome (proteinuria >3.5 g/day) or nephritic syndrome that has not improved with ACE inhibitor/ARB therapy 3

For Severe Renal Involvement (Crescentic HSP with Nephrotic Syndrome and/or Deteriorating Kidney Function)

• Treat with high-dose intravenous methylprednisolone plus cyclophosphamide, following the same protocol as crescentic IgA nephropathy 3, 1
• Cyclosporine may be considered as an alternative, though long-term outcomes are not significantly better than high-dose IV corticosteroids, and nephrotoxicity limits its use in relapsing disease 3
• Alternative immunosuppressive agents include azathioprine, tacrolimus, or mycophenolate mofetil/mycophenolic acid when cyclophosphamide is contraindicated due to side effects 1

Adult HSP Nephritis

• Treat adults with HSP nephritis using the same approach as children, though adults have worse prognosis and higher risk of progression to end-stage renal disease 3

Gastrointestinal and Joint Symptoms

• Use oral prednisone 1-2 mg/kg daily for 2 weeks for severe abdominal pain or joint symptoms 2, 4
• A meta-analysis found corticosteroids reduced mean time to resolution of abdominal pain in children 2
• Consider colchicine 1 mg/day for persistent purpura and pain, continuing for at least 6 months 1

What NOT to Do

• Do NOT use corticosteroids prophylactically at HSP onset to prevent nephritis—moderate quality evidence (Level 1B) shows no benefit in preventing nephritis or reducing risk of severe persistent nephritis 3, 1, 5
• Do NOT use antiplatelet agents—no significant difference in preventing persistent kidney disease 5
• Do NOT use heparin despite one study showing benefit, as the risk of bleeding is not justified when fewer than 2% of children with HSP develop severe kidney disease 5

Monitoring Protocol

• Obtain urinalysis with microscopy at presentation and regularly during follow-up to assess for proteinuria, red blood cell casts, and dysmorphic red blood cells 1
• Check basic metabolic panel (BUN, creatinine) and complete blood count with platelets at baseline 1
• Perform renal biopsy in children with decreased renal function at presentation or with severe nephrotic/nephritic syndrome 3
• Monitor for persistent hematuria and proteinuria as indicators of ongoing disease activity 1

Prognosis

• HSP spontaneously resolves in 94% of children and 89% of adults, with average disease duration of 4 weeks 2, 4
• End-stage renal disease occurs in 1-5% of patients, making renal involvement the most important prognostic factor 2, 4
• Long-term prognosis depends entirely on severity of renal involvement 2

Common Pitfalls

• Starting corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy 3
• Using NSAIDs for pain management, which can worsen renal function 1
• Failing to monitor blood pressure and urine regularly during follow-up 1
• Attempting to normalize proteinuria to <0.5 g/day/1.73 m², which increases side effects without proven benefit 3