What is the management of Henoch-Schonlein purpura (HSP)?

Management of Henoch-Schönlein Purpura (HSP)

The management of Henoch-Schönlein purpura should focus primarily on supportive care for mild cases, with specific interventions for organ involvement, particularly using ACE inhibitors or ARBs for nephritis with proteinuria, and corticosteroids for severe manifestations. 1

Clinical Presentation and Diagnosis

HSP is characterized by:

• Palpable purpura (present in 100% of patients)
• Joint symptoms (75% of patients)
• Gastrointestinal symptoms (60-65% of patients)
• Renal involvement (40-50% of patients) 1, 2

Diagnosis is based on the finding of palpable purpura with at least one of the following:

• Diffuse abdominal pain
• Arthritis or arthralgia
• Renal involvement (hematuria and/or proteinuria)
• Biopsy showing predominant IgA deposition 3

Management Approach

1. Supportive Care (Primary Management)

• HSP spontaneously resolves in 94% of children and 89% of adults 2
• Supportive treatment is the primary intervention for most cases
• Monitor for resolution of skin manifestations and improvement in abdominal and joint symptoms
• Pain management with NSAIDs for joint pain

2. Management of Specific Manifestations

Renal Involvement

• For persistent proteinuria:

  • ACE inhibitors or ARBs are recommended as first-line therapy 1
  • For proteinuria >1 g/day per 1.73 m² after ACE inhibitor/ARB trial and GFR >50 ml/min per 1.73 m², a 6-month course of corticosteroid therapy is recommended 1

• For severe nephritis (crescentic HSP with nephrotic syndrome and/or deteriorating kidney function):

  • Treatment as for crescentic IgA nephropathy with steroids and cyclophosphamide is suggested 1
  • However, evidence from RCTs shows no significant benefit of cyclophosphamide in children or adults with HSP and severe kidney disease 4

Gastrointestinal Symptoms

• Oral prednisone at 1-2 mg/kg daily for two weeks can be used to treat severe abdominal pain 2
• A meta-analysis found that corticosteroid use in children reduced the mean time to resolution of abdominal pain 2

Joint Symptoms

• Supportive care with analgesics
• Oral prednisone may help with severe arthritis 2

3. Prevention of Renal Disease

• Corticosteroids are NOT recommended for prevention of HSP nephritis 1
• Evidence shows no significant difference in the risk of persistent kidney disease in children given prednisone for 14-28 days at presentation compared with placebo or supportive treatment 4
• Antiplatelet therapy has shown no significant differences in the risk of persistent kidney disease 4

Monitoring Protocol

Based on the Alder Hey Henoch Schönlein Purpura Pathway 5:

• Six-month monitoring period for all patients presenting with HSP
• Prioritize monitoring based on urine findings on day 7:
  • Normal urinalysis on day 7 has a 97% negative predictive value for normal renal outcome 5
  • Patients with abnormal urinalysis require more intensive monitoring

Risk Factors for Severe Disease

• Older age is associated with higher risk of renal involvement 5
• Adults with HSP are more likely to experience complications than children 2
• End-stage renal disease occurs in 1-5% of patients 2

Long-term Prognosis

• Most cases are self-limited with an average duration of 4 weeks 3
• Long-term prognosis depends primarily on the severity of renal involvement 2, 4
• Long-term complications are rare and include persistent hypertension and end-stage kidney disease 3

Areas Requiring Further Research

• The role of immunosuppressants like cyclosporin and mycophenolate mofetil remains unclear due to small patient numbers in studies 4
• Further trials to clarify the role of glucocorticosteroids are needed before a definitive role for steroids in HSP management can be established 6