What is the current treatment approach for Henoch‑Schönlein purpura (IgA‑mediated small‑vessel vasculitis) in a typical 7‑year‑old child?

Treatment of Henoch-Schönlein Purpura (IgA Vasculitis) in Children

For a typical 7-year-old child with HSP, supportive care alone is the primary treatment strategy, as 94% of pediatric cases resolve spontaneously within 4 weeks; corticosteroids should NOT be used prophylactically but are reserved for severe gastrointestinal pain, gastrointestinal hemorrhage, or significant renal involvement with persistent proteinuria. 1, 2, 3

Initial Management: Supportive Care

The cornerstone of HSP treatment is supportive management, not immunosuppression. 2, 3, 4

• Provide adequate hydration and rest during the acute phase 4
• Use acetaminophen for pain relief; avoid NSAIDs and aspirin as they increase bleeding risk in the setting of vasculitis and potential renal involvement 4
• Monitor for complications through serial clinical assessment 2, 3
• Educate families that the average disease duration is 4 weeks, with most cases self-limiting 2

When Corticosteroids ARE Indicated

Corticosteroids have a specific, limited role in HSP and should only be used for:

• Severe abdominal pain that is refractory to supportive measures 1, 2, 5
• Gastrointestinal hemorrhage documented by melena, hematemesis, or positive fecal occult blood 2, 3
• Severe nephritis with proteinuria >1 g/day per 1.73 m² after trial of ACE inhibitors or ARBs 1
• Life-threatening complications such as pulmonary hemorrhage, central nervous system involvement, or testicular torsion 5, 3

Dose: Oral prednisone 1–2 mg/kg/day (maximum 60 mg/day) for 1–2 weeks, then taper over 1–2 weeks 2, 3

Critical Evidence: What Corticosteroids Do NOT Do

Systematic reviews and randomized trials demonstrate that prophylactic corticosteroids do NOT:

• Prevent the development of nephritis 1, 2, 3
• Reduce the severity of renal involvement 1, 2
• Shorten disease duration in uncomplicated cases 3, 4
• Prevent disease recurrence (which occurs in 30–40% regardless of steroid use) 2, 3

Therefore, routine steroid use in all HSP patients is not supported by current evidence. 1, 3

Management of Renal Involvement

For children with HSP nephritis, treatment is stratified by severity:

Mild Nephritis (Hematuria ± Proteinuria <0.5 g/day per 1.73 m²)

• Observation with monthly urinalysis and blood pressure monitoring for at least 6 months 1, 2, 3
• ACE inhibitors or ARBs if proteinuria persists beyond 3 months 1, 2

Moderate Nephritis (Proteinuria 0.5–1 g/day per 1.73 m²)

• ACE inhibitors or ARBs as first-line therapy 1, 2
• Add corticosteroids if proteinuria persists after 3–6 months of ACE inhibitor/ARB therapy 1

Severe Nephritis (Proteinuria >1 g/day per 1.73 m², Nephrotic Syndrome, or Crescentic Glomerulonephritis)

• Treat the same as IgA nephropathy: 6-month course of corticosteroid therapy (prednisone 1–2 mg/kg/day for 2 months, then taper over 4 months) 1
• For crescentic HSP nephritis with rapidly progressive renal deterioration (>50% crescents on biopsy): Use corticosteroids plus cyclophosphamide, analogous to ANCA vasculitis treatment 1, 5, 3
• Consider mycophenolate mofetil or cyclosporine for steroid-dependent or steroid-resistant cases 5, 6, 3

Second-Line and Rescue Therapies

For rare, life-threatening complications or severe refractory disease:

• Methylprednisolone pulse therapy (30 mg/kg IV daily for 3 days, maximum 1 g/day) for severe gastrointestinal bleeding, pulmonary hemorrhage, or central nervous system vasculitis 5, 3
• Cyclophosphamide (2 mg/kg/day oral or 500–750 mg/m² IV monthly) for crescentic nephritis or life-threatening organ involvement 1, 5
• Cyclosporine A (3–5 mg/kg/day in divided doses) for steroid-dependent cases 6
• Mycophenolate mofetil (600 mg/m² twice daily) for severe nephritis 3
• Plasma exchange for rapidly progressive glomerulonephritis unresponsive to steroids and cyclophosphamide 5
• IVIG (1–2 g/kg) for refractory cases, though evidence is limited 5

Monitoring and Follow-Up Strategy

All children with HSP require:

• Weekly urinalysis and blood pressure checks during the acute phase (first 4–6 weeks) 2, 3
• Monthly urinalysis and blood pressure monitoring for at least 6 months after symptom resolution, as renal involvement can appear late or relapse 2, 3
• Immediate renal biopsy if nephrotic-range proteinuria (>40 mg/m²/hour or protein:creatinine ratio >2), rapidly rising creatinine, or nephritic syndrome develops 1, 2
• Long-term annual follow-up for patients who had any degree of nephritis, as chronic kidney disease can develop years later 2, 3

Common Pitfalls to Avoid

• Do not start corticosteroids in every child with HSP – this exposes 90% of patients to unnecessary steroid toxicity without benefit 1, 2, 3
• Do not assume steroids prevent nephritis – early steroid treatment does not reduce the incidence or severity of renal involvement 1, 2
• Do not discharge without arranging urinalysis follow-up – renal involvement can appear weeks after initial presentation and is the primary determinant of long-term morbidity 2, 3
• Do not use NSAIDs for pain control – they worsen renal perfusion and increase bleeding risk in the setting of vasculitis 4
• Do not miss gastrointestinal complications – intussusception, bowel perforation, and massive hemorrhage can occur and require surgical consultation 2, 3

Prognosis and Long-Term Outcomes

• Excellent prognosis in most cases: 94% of children achieve complete spontaneous resolution 3
• Renal involvement is the most important prognostic factor: approximately 2% of children with HSP progress to end-stage renal disease, and up to 20% of those with severe nephritis may require dialysis 2, 5
• Recurrence is common: 30–40% of children experience at least one recurrence, typically within 4–6 months of initial presentation 2, 3
• Adult-onset HSP has worse renal prognosis: up to 11% of adults progress to chronic kidney disease compared to 2% of children 5, 3