What is the recommended management for Henoch-Schönlein Purpura (HSP) in children?

Management of Henoch-Schönlein Purpura (HSP) in Children

Most cases of Henoch-Schönlein Purpura in children are self-limited and require only supportive care, but specific interventions are needed for organ involvement, particularly renal disease which is the most important prognostic factor determining long-term morbidity and mortality. 1

Initial Assessment and Diagnosis

• Diagnosis based on palpable purpura plus at least one of:

  • Diffuse abdominal pain
  • Arthritis or arthralgia
  • Renal involvement (hematuria and/or proteinuria)
  • Biopsy showing predominant IgA deposition 1

• Peak incidence: 4-7 years of age (90% of cases occur in children 2-10 years) 1

• Average duration: 4 weeks 1

• Seasonal variation: More common in autumn, winter, and spring 2

• Often follows upper respiratory tract infection 2

Treatment Approach by Organ System

General Management

• Most cases resolve with supportive care alone 1
• Adequate hydration
• Pain control with appropriate analgesics
• Rest during acute phase

Skin Manifestations

• Typically self-limiting
• No specific treatment required for the rash itself

Joint Involvement (58.1% of cases) 3

• NSAIDs (e.g., ibuprofen) for arthralgia/arthritis unless contraindicated by GI or renal involvement

Gastrointestinal Involvement (56% of cases) 3

• Mild symptoms: Supportive care
• Severe GI pain or hemorrhage: Consider oral corticosteroids 1
• Monitor for intussusception, which is associated with higher rates of renal involvement 3
• Note: Severe GI involvement is associated with higher risk of renal involvement and nephritis 3

Renal Involvement (29.8% of cases) 3

• For HSP nephritis with proteinuria persisting >3 months: ACE inhibitors or ARBs should be considered in addition to corticosteroids 1
• For severe nephritis:
  1. Methylprednisolone pulse therapy followed by oral prednisone 4
  2. For resistant cases: Consider cyclophosphamide (demonstrated effective in randomized controlled trials) 4
  3. Other options with less evidence: Cyclosporin A, azathioprine, mycophenolate mofetil 4
  4. Plasmapheresis may be beneficial in delaying progression of kidney disease in severe cases 4

Medication Options

Corticosteroids

• Not recommended for universal prophylactic treatment 1
• Indicated for:
  • Severe gastrointestinal pain
  • Gastrointestinal hemorrhage
  • Severe nephritis

Immunosuppressants

• Cyclophosphamide: Most evidence for efficacy in severe HSP nephritis 4
• Cyclosporin A: May be used in combination with steroids for nephritis 4
• Other agents (limited evidence): Azathioprine, mycophenolate mofetil 4

Other Medications

• Colchicine: May be effective for relapsing disease (showed favorable response in 11 of 12 relapsing patients who did not respond to ibuprofen or steroids) 3
• ACE inhibitors/ARBs: For persistent proteinuria to prevent/limit secondary glomerular injury 1

Follow-up Recommendations

• All children with HSP should be monitored for at least 6 months 5
• Follow-up should include:
  • Regular urine testing for proteinuria and hematuria
  • Blood pressure measurement 5
• Long-term follow-up for those with renal involvement
• Women with childhood HSP require close monitoring during pregnancy due to increased risk of complications (proteinuria and hypertension) 5

Prognosis

• Most cases have excellent outcomes 1
• Renal involvement is the most important prognostic factor 1
• Long-term complications are rare but include:
  • Persistent hypertension
  • End-stage kidney disease 1

Special Considerations

• Relapse occurs in approximately 16.4% of patients 3
• For relapsing cases unresponsive to ibuprofen or steroids, colchicine may be effective 3
• Early steroid treatment does not reduce the incidence or severity of nephropathy 1