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

Treatment of Henoch-Schönlein Purpura (HSP)

Most HSP cases are self-limited and require only supportive care, but specific organ involvement—particularly renal disease—demands targeted pharmacologic intervention with ACE inhibitors/ARBs as first-line therapy, followed by corticosteroids for severe nephritis. 1

General Management Principles

The majority of HSP cases (94% in children, 89% in adults) resolve spontaneously within 4 weeks, making supportive care the primary intervention. 2, 3

• Acetaminophen (paracetamol) is the preferred analgesic for pain management, as NSAIDs like ketorolac (Toradol) carry risk of acute kidney injury, especially given the frequent renal involvement in HSP 1
• Monitor blood pressure at every visit, as hypertension indicates more severe renal involvement 1
• Perform urinalysis with microscopy at diagnosis and regularly during follow-up to detect proteinuria, red blood cell casts, and dysmorphic red blood cells 1

Treatment Based on Organ Involvement

Cutaneous and Joint Manifestations

• For isolated purpura and arthralgia without severe symptoms, no specific pharmacologic treatment is required beyond acetaminophen for pain relief 1, 2
• Oral prednisone 1-2 mg/kg daily for two weeks may be used for severe joint pain and widespread cutaneous flares, though this does not prevent nephritis development 1, 2
• Colchicine 1 mg/day for at least 6 months can be considered for persistent purpura and pain 1

Gastrointestinal Involvement

• Oral corticosteroids (prednisone 1-2 mg/kg daily for two weeks) should be considered for severe abdominal pain and gastrointestinal hemorrhage, as meta-analysis shows reduced time to resolution of abdominal symptoms 2, 3
• Current evidence does not support universal corticosteroid treatment for all HSP patients—reserve for symptomatic gastrointestinal disease 3

Renal Disease Management (Most Critical for Long-Term Prognosis)

Renal involvement determines morbidity and mortality in HSP, occurring in 40-50% of patients, with worse prognosis in adults. 2, 4

Mild to Moderate Nephritis (Persistent Proteinuria)

• Start ACE inhibitor or ARB therapy for persistent proteinuria, even though evidence is extrapolated from IgA nephropathy rather than HSP-specific trials 1
• Target proteinuria to <1 g/day/1.73 m² rather than attempting complete normalization, which increases side effects without proven benefit 1
• This applies equally to children and adults 1

Moderate Nephritis (Proteinuria >1 g/day/1.73 m² After ACE/ARB Trial)

• Add a 6-month course of corticosteroid therapy if proteinuria persists >1 g/day/1.73 m² after ACE inhibitor/ARB trial AND GFR remains >50 ml/min/1.73 m² 1
• Reserve corticosteroids for nephrotic syndrome (proteinuria >3.5 g/day) or nephritic syndrome unresponsive to ACE inhibitor/ARB therapy 1

Severe Nephritis (Crescentic HSP with Nephrotic Syndrome and/or Deteriorating Kidney Function)

• Treat with high-dose intravenous methylprednisolone PLUS cyclophosphamide, following the same protocol as crescentic IgA nephropathy 1, 4
• Alternative immunosuppressive agents include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid when cyclophosphamide is contraindicated due to side effects 1
• However, one randomized trial in adults showed adding cyclophosphamide to steroids provided no benefit compared with steroids alone for severe HSP 5
• Plasma exchange and polyclonal immunoglobulin therapy are reserved for rare life-threatening forms 4

Critical Pitfalls to Avoid

• Do NOT use corticosteroids prophylactically at HSP onset to prevent nephritis—moderate quality evidence (Level 1B) shows no benefit in preventing nephritis or reducing risk of severe persistent nephritis 1
• Do NOT use NSAIDs (including ketorolac/Toradol) in HSP patients due to risk of acute kidney injury in the setting of potential renal involvement 1
• Do NOT start corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy, as this increases side effects without proven benefit 1
• Do NOT attempt to normalize proteinuria to <0.5 g/day/1.73 m², as this increases side effects without proven benefit 1

Monitoring Protocol

• Perform urinalysis with microscopy at diagnosis and at regular intervals to detect persistent hematuria and proteinuria, which indicate ongoing disease activity 1
• Measure blood pressure at every visit 1
• Check basic metabolic panel including BUN and serum creatinine to assess renal function 1
• Renal ultrasound is the preferred initial imaging modality if renal biopsy is being considered for severe nephritis 1

Long-Term Prognosis

• End-stage renal disease occurs in 1-5% of patients, with renal involvement being the most important prognostic factor 2, 3
• Adults have worse prognosis and higher risk of progression to end-stage renal disease compared to children 1, 4
• Up to 20% of children with nephritis treated in specialized centers may require hemodialysis 4

Adjunctive Dietary Considerations

• 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