Management of Henoch-Schönlein Purpura in Children
Initial Supportive Care is the Foundation
For the typical child with HSP, observation with supportive measures alone is the appropriate management, as 94% resolve spontaneously within 4 weeks without intervention. 1
Core Supportive Measures
- Adequate hydration and rest during the acute phase 2
- Analgesics (acetaminophen or ibuprofen) for joint pain and mild abdominal discomfort 2
- Avoid aspirin and antiplatelet agents, as HSP does not cause thrombocytopenia and these agents increase bleeding risk 2
- Activity restriction is not necessary once acute symptoms resolve 1
When NOT to Use Corticosteroids
Prophylactic corticosteroids should never be given to prevent nephritis or other complications, as systematic reviews demonstrate they do not prevent renal involvement, do not reduce renal severity, do not shorten disease duration, and do not prevent recurrence. 1, 3, 4
This is a critical pitfall: the natural history shows 30–40% recurrence regardless of steroid use, and early steroid treatment does not alter this trajectory 1, 2.
Indications for Corticosteroid Therapy
Corticosteroids are reserved for specific complications only 1:
Severe Abdominal Pain or GI Hemorrhage
- Prednisone 1–2 mg/kg/day (maximum 60 mg) for 1–2 weeks, followed by a taper over 1–2 weeks 1, 2
- Randomized trials show oral corticosteroids reduce severe gastrointestinal pain and bleeding within 24–48 hours 4
- Do not use steroids for mild abdominal discomfort that responds to supportive care 1
Life-Threatening Complications
- Pulmonary hemorrhage, CNS vasculitis, or testicular torsion require methylprednisolone pulse therapy (30 mg/kg IV daily × 3 days) 1
- These presentations are rare but carry high morbidity if untreated 5
Renal Involvement: Monitoring and Treatment Algorithm
Surveillance Strategy
- Weekly urinalysis and blood pressure checks during the first 4–6 weeks 1
- Monthly urinalysis and blood pressure for at least 6 months after symptom resolution 1
- Annual long-term follow-up for any child who experienced nephritis, as chronic kidney disease can develop years later 1
Mild Nephritis (Hematuria ± Proteinuria <0.5 g/day per 1.73 m²)
- Observe with monthly urinalysis and blood pressure monitoring for ≥6 months 1
- Initiate an ACE inhibitor or ARB if proteinuria persists beyond 3 months 1, 2
Moderate Nephritis (Proteinuria 0.5–1 g/day per 1.73 m²)
- Start ACE inhibitor or ARB as first-line therapy 1
- Add corticosteroids only if proteinuria remains after 3–6 months of ACE/ARB therapy 1
Severe Nephritis (Proteinuria >1 g/day per 1.73 m², Nephrotic Syndrome, or Crescentic GN)
- Treat with a 6-month corticosteroid course: prednisone 1–2 mg/kg/day for 2 months, then taper over 4 months 1
- For crescentic HSP nephritis with >50% crescents and rapid renal decline, combine corticosteroids with cyclophosphamide (2 mg/kg/day oral or 500–750 mg/m² IV monthly) 1, 5
- Immediate renal biopsy is required if nephrotic-range proteinuria, rapidly rising creatinine, or nephritic syndrome develop 1
Steroid-Dependent or Steroid-Resistant Cases
- Consider mycophenolate mofetil (evidence from case reports shows efficacy in severe gastrointestinal and renal HSP) 6, 4
- Cyclosporine is an alternative second-line agent 4
- Plasma exchange for rapidly progressive glomerulonephritis unresponsive to steroids and cyclophosphamide 1, 5
Critical Pitfalls to Avoid
- Never treat based on purpura alone—the rash is benign and does not require therapy 1
- Never use prolonged corticosteroids in children due to growth suppression, immunosuppression, and metabolic effects 2
- Never assume HSP is benign without renal surveillance—up to 20% of children with severe nephritis may require dialysis, and 2% progress to end-stage renal disease 1, 5
- Never discharge a child with HSP without arranging follow-up urinalysis—renal involvement can appear weeks after initial presentation 1
Prognosis and Recurrence
- 94% of children achieve complete spontaneous resolution 1, 4
- Recurrence occurs in 30–40% of children, typically within 4–6 months, independent of steroid use 1
- Renal involvement is the key prognostic factor: ≈2% progress to end-stage renal disease, and up to 20% of those with severe nephritis may require dialysis 1, 5