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

Management of Henoch-Schönlein Purpura (HSP)

HSP is primarily a self-limited disease requiring supportive care, with corticosteroids reserved for severe gastrointestinal or joint symptoms, and ACE inhibitors/ARBs plus corticosteroids for significant renal involvement. 1

Initial Assessment and Diagnosis

Diagnose HSP based on palpable purpura plus at least one of the following: renal involvement (hematuria/proteinuria), diffuse abdominal pain, arthritis/arthralgia, or biopsy showing predominant IgA deposition. 1, 2

Key clinical features to document:

• Purpuric rash (present in 100% of cases) 3, 4
• Arthritis/arthralgia (58-82% of cases) 3, 4, 5
• Abdominal pain (56-65% of cases) 3, 4, 5
• Renal involvement (30-50% of cases) 3, 4, 5, 2

Supportive Management (First-Line for Most Patients)

The disease spontaneously resolves in 94% of children and 89% of adults, making supportive care the primary intervention. 3

• Provide symptomatic pain relief with acetaminophen (paracetamol) as the safer first-line analgesic option 1
• Avoid NSAIDs like ketorolac (Toradol) due to risk of acute kidney injury, especially with pre-existing renal impairment 1
• Ensure adequate hydration and rest 6
• Monitor for complications during the acute phase (average disease duration is 4 weeks) 2

Treatment of Gastrointestinal and Joint Symptoms

For severe abdominal pain or joint symptoms, use oral prednisone 1-2 mg/kg daily for two weeks. 1, 3, 4

• Corticosteroids hasten resolution of arthritis and abdominal pain but do not prevent recurrences 4
• A meta-analysis found corticosteroids reduced mean time to resolution of abdominal pain in children 3
• For persistent purpura and pain, consider colchicine 1 mg/day for at least six months 1
• Colchicine may be effective in relapsing patients who do not respond to ibuprofen or steroids 5

Renal Disease Management (Critical for Long-Term Prognosis)

The long-term prognosis depends entirely on the severity of renal involvement, with end-stage renal disease occurring in 1-5% of patients. 3

Mild Renal Involvement (Hematuria/Mild Proteinuria)

• Start ACE inhibitors or ARBs for children with HSP nephritis and persistent proteinuria 1
• Monitor blood pressure, urinalysis, and renal function regularly 2

Moderate Renal Involvement (Persistent Proteinuria >1 g/day per 1.73 m²)

• After trial of ACE inhibitor/ARB, if proteinuria persists and GFR >50 ml/min per 1.73 m², initiate a 6-month course of corticosteroid therapy 1
• Consider ACE inhibitor/ARB continuation to prevent secondary glomerular injury 2

Severe Renal Involvement (Crescentic HSP with Nephrotic Syndrome/Deteriorating Function)

• Treat with steroids plus cyclophosphamide 1
• Alternative immunosuppressants include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid 1
• High-dose intravenous pulse methylprednisolone coupled with azathioprine or cyclophosphamide may benefit patients with severe nephritis 4

Adults with HSP Nephritis

• Use the same treatment approach as in children 1

Prevention of Renal Complications

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

• Early corticosteroid treatment does not reduce the incidence or severity of nephropathy in children with HSP 2
• Focus on early detection and treatment of established renal disease rather than prevention 1

Management of Recurrent Disease

Disease recurs at least once in 16.4% of patients. 5

• Corticosteroids do not prevent recurrences 4
• Colchicine treatment yields favorable response in relapsing patients who do not respond to other therapies 5
• Continue monitoring for 6-12 months after initial presentation, as recurrences can occur 6

Dietary Considerations (Adjunctive)

A low-antigen-content (LAC) diet may improve symptoms and laboratory abnormalities when strictly followed for 4-8 weeks, and can be considered as supportive treatment in conjunction with other therapies. 1

Monitoring and Follow-Up

All HSP patients require long-term renal monitoring regardless of initial presentation. 2

• Monitor urinalysis and blood pressure for at least 6 months after diagnosis 2
• Patients with any degree of renal involvement require extended follow-up to detect late-onset chronic kidney disease 3
• Watch for intussusception in patients with severe gastrointestinal involvement, as it correlates with higher rates of renal involvement 5

Common Pitfalls to Avoid

• Do not use NSAIDs for pain control due to nephrotoxicity risk 1
• Do not prescribe prophylactic corticosteroids to prevent nephritis 1
• Do not assume corticosteroids will prevent disease recurrence 4
• Do not delay aggressive immunosuppression in patients with crescentic nephritis and deteriorating renal function 1
• Do not discontinue renal monitoring after acute phase resolution, as complications can develop later 2