What is the management of Henoch-Schönlein Purpura (HSP)?

Management of Henoch-Schönlein Purpura (HSP)

Most HSP cases are self-limited and require only supportive care, with corticosteroids reserved for severe gastrointestinal or joint symptoms, while renal involvement demands ACE inhibitors/ARBs as first-line therapy and corticosteroids for persistent proteinuria >1 g/day. 1

Initial Assessment and Diagnosis

Diagnostic Criteria:

• Diagnosis requires palpable purpura plus at least one of: renal involvement (hematuria/proteinuria), diffuse abdominal pain, arthritis/arthralgia, or biopsy showing predominant IgA deposition 1, 2
• All patients develop purpuric rash, 75% develop arthritis, 60-65% develop abdominal pain, and 40-50% develop renal disease 3

General Management Approach

Supportive Care (First-Line for Most Patients):

• HSP spontaneously resolves in 94% of children and 89% of adults, making supportive treatment the primary intervention 3
• Average disease duration is 4 weeks 2
• Management focuses on symptom relief and monitoring for complications 4

Organ-Specific Management

Gastrointestinal and Joint Symptoms

Corticosteroid Use:

• Oral prednisone 1-2 mg/kg daily for 2 weeks may be used for severe abdominal pain and joint symptoms 1, 3
• Corticosteroids reduce mean time to resolution of abdominal pain and may decrease odds of developing persistent renal disease in children 3
• Important caveat: Current evidence does NOT support universal corticosteroid treatment for all HSP patients 2
• Consider corticosteroids specifically for severe gastrointestinal pain and gastrointestinal hemorrhage 2

Alternative for Persistent Symptoms:

• Colchicine 1 mg/day may be considered for persistent purpura and pain, with treatment for at least 6 months 1

Renal Disease Management (Critical for Long-Term Prognosis)

Mild Renal Involvement:

• For persistent proteinuria, initiate ACE inhibitors or ARBs as first-line therapy 1
• This applies to both children and adults 1

Moderate Renal Involvement:

• For persistent proteinuria >1 g/day per 1.73 m² after ACE inhibitor/ARB trial AND GFR >50 ml/min per 1.73 m², add a 6-month course of corticosteroid therapy 1

Severe Renal Involvement:

• For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treat with steroids PLUS cyclophosphamide 1, 5
• High-dose corticosteroids (IV pulse methylprednisolone or oral prednisone) combined with oral cyclophosphamide 2 mg/kg/day for 12 weeks has shown significant reduction in proteinuria 5
• Alternative immunosuppressants when needed include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid 1

Important Note on Cyclophosphamide in Adults:

• In adults with severe HSP, adding cyclophosphamide to steroids provides no additional benefit compared to steroids alone, based on a prospective trial 6
• This contrasts with the pediatric approach where combination therapy is recommended for crescentic disease 1

Prophylaxis

What NOT to Do:

• Prophylactic corticosteroids are NOT recommended to prevent HSP nephritis (strong evidence) 1

Pain Management Considerations

Analgesic Selection:

• Acetaminophen (paracetamol) is the safer first-line analgesic option 1
• Avoid NSAIDs like ketorolac (Toradol): These can cause acute kidney injury, especially problematic given the high incidence of renal involvement in HSP 1

Monitoring and Follow-Up

Renal Monitoring:

• Monitor for hypertension development during treatment 5
• Long-term prognosis depends on severity of renal involvement 3
• End-stage renal disease occurs in 1-5% of patients 3

Duration of Follow-Up:

• Long-term complications are rare but include persistent hypertension and end-stage kidney disease 2
• Renal involvement is the most important prognostic factor determining morbidity and mortality 2

Supportive Dietary Measures

Low-Antigen-Content (LAC) Diet:

• May improve symptoms and laboratory abnormalities when strictly followed for 4-8 weeks 1
• Can be used in conjunction with other treatments like colchicine for chronic or persistent symptoms 1

Common Pitfalls to Avoid

• Do not universally prescribe corticosteroids for all HSP patients—reserve for specific indications (severe GI symptoms, severe renal involvement) 2
• Do not use NSAIDs for pain control due to nephrotoxicity risk in a disease with high renal involvement 1
• Do not add cyclophosphamide to steroids in adults with severe HSP unless there is crescentic nephritis, as it provides no additional benefit 6
• Do not give prophylactic steroids to prevent nephritis—this is not effective 1