What is the management and treatment approach for a child or young adult diagnosed with Henoch-Schonlein purpura (HSP)?

Management of Henoch-Schönlein Purpura in Children and Young Adults

For most children with HSP, supportive care alone is sufficient, with corticosteroids reserved only for severe gastrointestinal pain or established severe nephritis—never for prevention of kidney disease. 1

Initial Assessment and Diagnosis

Confirm the diagnosis when palpable purpura is present plus at least one of: renal involvement (hematuria/proteinuria), arthralgia/arthritis, or abdominal pain. 2, 3 The triad of hematuria, purpuric lesions, and ankle pain is specifically diagnostic. 2, 3

Obtain baseline studies immediately:

• Urinalysis with microscopy looking for red blood cell casts, dysmorphic RBCs, and proteinuria 2, 3
• Quantify proteinuria (spot urine protein/creatinine ratio or 24-hour collection) 2, 3
• Basic metabolic panel (BUN, creatinine) 2, 3
• Complete blood count with platelets to rule out thrombocytopenia 2
• Blood pressure measurement (hypertension indicates severe renal involvement) 2, 3

Treatment Algorithm Based on Clinical Presentation

For Uncomplicated HSP (No Renal Involvement)

Provide supportive care only:

• Use acetaminophen for pain control—never NSAIDs like ketorolac (Toradol) as they cause acute kidney injury in HSP patients 2, 3
• Monitor with urinalysis and blood pressure at least every 2 weeks for 6 months 4, 5
• Do not use prophylactic corticosteroids—moderate quality evidence (Level 1B) shows no benefit in preventing nephritis 1, 2, 3

For Severe Gastrointestinal Symptoms

Consider oral prednisone 1-2 mg/kg/day for 2 weeks if severe abdominal pain or GI hemorrhage is present. 2, 6 This addresses pain control but does not prevent renal complications. 6

For Mild Renal Involvement (Microscopic Hematuria, Proteinuria 0.5-1 g/day/1.73 m²)

Start ACE inhibitor or ARB therapy targeting proteinuria to <1 g/day/1.73 m². 2, 3 This is extrapolated from IgA nephropathy data but represents the standard approach. 2 Do not attempt to normalize proteinuria to <0.5 g/day as this increases side effects without proven benefit. 2

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

If GFR >50 ml/min/1.73 m² and proteinuria persists >1 g/day after adequate ACE-I/ARB trial, add a 6-month course of oral corticosteroids. 1, 2, 3 The evidence for this approach is limited, but it represents the consensus recommendation. 1

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

Treat aggressively with high-dose intravenous methylprednisolone plus cyclophosphamide, following the same protocol as crescentic IgA nephropathy. 1, 2, 3 This is the only scenario where cyclophosphamide has evidence of benefit based on a single RCT. 1

Alternative immunosuppressive agents (cyclosporine, tacrolimus, mycophenolate mofetil, azathioprine) may be considered, but evidence is weaker. 1 Cyclosporine may be effective for heavy proteinuria and crescentic HSP, though nephrotoxicity limits its use in relapse. 1

Perform renal biopsy if there is decreased renal function at presentation, severe nephrotic syndrome (proteinuria >3.5 g/day), or deteriorating kidney function. 3

Special Populations

Adults with HSP Nephritis

Treat adults using the same approach as children, though adults have worse prognosis with higher risk of progression to end-stage renal disease. 1, 2 Adults are more likely to have severe initial renal involvement and protein excretion >1 g/day predicts worse outcomes. 1

Women with History of Childhood HSP

Monitor closely during pregnancy as they are at increased risk of proteinuria and hypertension complications. 4

Critical Pitfalls to Avoid

Never use corticosteroids prophylactically at HSP onset—this is a Level 1B (strong) recommendation against prophylaxis as it does not prevent nephritis or reduce risk of severe persistent nephritis. 1, 2, 3

Never use NSAIDs for pain control in HSP patients with any renal involvement—use acetaminophen as first-line analgesic. 2, 3

Do not start corticosteroids for mild proteinuria without an adequate trial of ACE-I/ARB therapy first, as this increases side effects without proven benefit. 2

Do not attempt to normalize proteinuria to <0.5 g/day/1.73 m²—target <1 g/day instead to avoid unnecessary medication side effects. 2

Monitoring Protocol

All HSP patients require at least 6 months of monitoring with regular urinalysis and blood pressure checks, even if initial presentation had no renal involvement. 4, 5 A normal urinalysis on day 7 has a 97% negative predictive value for normal renal outcome, but monitoring must continue. 5

Intensify monitoring for patients with abnormal urine findings on day 7, as they are at higher risk of developing nephritis. 5 Older patients (mean age 12.3 years) are at higher risk of requiring renal referral compared to younger patients (mean age 6.0 years). 5

Prognosis

Most cases are self-limited with an average disease duration of 4 weeks. 6 Approximately 90% of cases occur in children aged 2-10 years with peak incidence at 4-7 years. 6 Long-term complications are rare but include persistent hypertension and end-stage kidney disease, with renal involvement being the most important prognostic factor. 6