Management of Henoch-Schönlein Purpura
For most children with HSP, supportive care alone is sufficient as the disease is self-limited, but you must aggressively monitor and treat renal involvement with ACE inhibitors/ARBs as first-line therapy, escalating to corticosteroids only for persistent nephrotic-range proteinuria or crescentic disease, while avoiding prophylactic steroids which provide no benefit.
Initial Diagnostic Evaluation
Perform urinalysis with microscopy at every visit to detect hematuria, proteinuria, red blood cell casts, and dysmorphic red blood cells indicating glomerular involvement 1, 2. This is critical as failing to perform urinalysis at each visit can lead to delayed detection of renal complications 1.
Obtain the following baseline studies:
- Complete blood count with platelets to confirm non-thrombocytopenic purpura 2
- Basic metabolic panel (BUN, creatinine) to assess renal function 2
- Blood pressure measurement as hypertension indicates more severe renal involvement 2
- Renal ultrasound if considering biopsy for severe nephritis 2
The diagnosis is clinical: palpable purpura plus at least one of the following: diffuse abdominal pain, arthritis/arthralgia, renal involvement (hematuria/proteinuria), or biopsy showing predominant IgA deposition 2, 3.
Management Algorithm by Disease Severity
Mild HSP Without Significant Renal Involvement
Use acetaminophen for pain control as first-line analgesic 2. Never use NSAIDs (including ketorolac/Toradol) due to risk of acute kidney injury, especially with any degree of renal impairment 1, 2.
For joint pain and cutaneous symptoms, consider oral prednisone 1-2 mg/kg daily for two weeks 1, 2. For persistent purpura and pain, colchicine 1 mg/day for at least six months may be beneficial 1.
Do NOT use corticosteroids prophylactically at HSP onset to prevent nephritis—this has Level 1B evidence showing no benefit in preventing nephritis or reducing risk of severe persistent disease 4, 1, 2.
HSP with Persistent Proteinuria (Mild to Moderate Renal Involvement)
Start ACE inhibitor or ARB therapy for persistent significant proteinuria as first-line renal protection 4, 1, 2. This recommendation is extrapolated from IgA nephropathy data but represents the standard of care 4.
Target proteinuria to <1 g/day/1.73 m² rather than attempting complete normalization to <0.5 g/day/1.73 m², which increases medication side effects without proven benefit 4, 1, 2.
Maintain blood pressure below the 90th percentile for age and gender 5.
HSP with Nephrotic-Range Proteinuria
Reserve corticosteroid therapy for nephrotic syndrome (proteinuria >3.5 g/day) or nephritic syndrome that has not improved after 3-6 months of optimized ACE inhibitor/ARB therapy 4, 1.
When proteinuria persists >1 g/day per 1.73 m² despite ACE inhibitor/ARB trial and GFR remains >50 ml/min per 1.73 m², add a 6-month course of corticosteroid therapy 4, 1, 2.
Do NOT start corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy, as this increases side effects without proven benefit 1, 2.
Crescentic HSP with Severe Renal Involvement
Treat crescentic HSP (>50% of glomeruli with crescents) with nephrotic syndrome and/or deteriorating kidney function the same as crescentic IgA nephropathy: high-dose intravenous methylprednisolone plus cyclophosphamide 4, 1.
This represents the only scenario where cyclophosphamide has evidence of benefit, based on a single RCT 4.
Cyclosporine may be effective for heavy proteinuria and crescentic HSP, though long-term outcomes are not significantly better than high-dose IV corticosteroids, and nephrotoxicity limits its use in relapse 4.
Management of Gastrointestinal Complications
Oral corticosteroids may be considered for severe gastrointestinal pain and gastrointestinal hemorrhage 2, 3. However, this does not prevent GI complications when used prophylactically 6.
Be vigilant for severe complications including intussusception, gastritis, duodenitis, ileitis, ulcers, and bleeding from sites like Meckel's diverticulum 7.
Special Populations
Adults with HSP Nephritis
Treat adults with HSP nephritis using the same approach as children 4, 1, 2. However, recognize that adults have worse prognosis with proteinuria >1 g/day and higher risk of progression to end-stage renal disease 4, 2.
Therapies NOT Recommended
Mycophenolate mofetil showed no demonstrated benefit in preventing persistent kidney disease 8.
Antiplatelet agents have no significant benefit in preventing persistent kidney disease 8.
Heparin, while showing reduced risk of persistent kidney disease in one study, is not justified given that fewer than 2% of children with HSP develop severe kidney disease, and the bleeding risk is substantial 8.
Monitoring Protocol
Perform urinalysis at every clinical visit for at least 6 months to assess for disease relapse or remission 1, 6. Monitor for persistent hematuria and proteinuria as indicators of ongoing disease activity 1.
Renal biopsy should be performed in children with decreased renal function at presentation or with severe nephrotic/nephritic syndrome 4.
Critical Pitfalls to Avoid
- Never use prophylactic corticosteroids to prevent HSP nephritis—strong evidence shows no benefit 4, 1, 2
- Avoid NSAIDs entirely due to nephrotoxicity risk 1, 2
- Do not attempt to normalize proteinuria to <0.5 g/day/1.73 m²—this increases side effects without benefit 4, 1, 2
- Do not start corticosteroids before adequate trial of ACE inhibitor/ARB for mild-moderate proteinuria 1, 2
- Do not fail to perform urinalysis at every visit—this leads to delayed detection of complications 1
Prognosis
Most cases are self-limited with average disease duration of 4 weeks 3. HSP spontaneously resolves in 94% of children and 89% of adults 6. Long-term complications are rare but include persistent hypertension and end-stage kidney disease 3. Renal involvement is the most important prognostic factor determining morbidity and mortality 3, 9.