Management Approach for Henoch-Schönlein Purpura (HSP)
The management of Henoch-Schönlein Purpura should be primarily supportive for most cases, with specific organ-targeted interventions reserved for patients with severe manifestations, particularly those with significant renal involvement. 1, 2
Diagnosis and Initial Assessment
- HSP diagnosis is based on finding palpable purpura plus at least one of: renal involvement (hematuria/proteinuria), abdominal pain, or arthritis 1
- Most cases (90%) occur in children between 2-10 years of age, with peak incidence at 4-7 years 3
- Evaluate for potential triggers, which are identified in approximately 50% of cases (infections, medications, toxins) 4
General Management Principles
- Supportive care is the primary intervention as HSP spontaneously resolves in 94% of children and 89% of adults 2
- Monitor for complications affecting major organ systems (skin, joints, gastrointestinal tract, kidneys) 3
- Regular urinalysis to detect renal involvement, which occurs in 40-50% of patients 2
Specific Symptom Management
Pain Management
- Acetaminophen (paracetamol) is recommended as first-line analgesic due to safety profile 1
- Avoid NSAIDs such as ketorolac (Toradol) due to risk of acute kidney injury, especially with pre-existing renal impairment 1
- For joint pain and abdominal symptoms, oral prednisone at 1-2 mg/kg daily for two weeks may be beneficial 2
Skin Manifestations
- Primarily supportive care as cutaneous manifestations are self-limiting 3
- In adults with chronic cutaneous forms, dapsone may have beneficial effects 4
Gastrointestinal Symptoms
- Corticosteroids may be considered for severe gastrointestinal pain and hemorrhage 3
- Oral prednisone can reduce mean time to resolution of abdominal pain 2
Renal Disease Management
- 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 suggested 1
- For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treatment with steroids and cyclophosphamide is recommended 1
- Prophylactic use of corticosteroids is not recommended to prevent HSP nephritis 1
Severe Disease Management
- Early aggressive therapy with high-dose steroids plus immunosuppressants is recommended for patients with severe renal involvement 2
- For life-threatening forms or compromised renal function, consider methylprednisolone pulse therapy, immunosuppressive drugs, plasma exchange, or polyclonal immunoglobulin therapy 4
- Addition of cyclophosphamide to steroids has not shown benefit compared to steroids alone in adults with severe HSP 5
Special Considerations
Adults vs. Children
- Adults with HSP are more likely to experience complications than children 2
- Renal prognosis appears to be worse in adults 4
- Treatment approach for adults with HSP nephritis should be the same as in children 1
Prognosis and Follow-up
- Long-term prognosis depends primarily on the severity of renal involvement 2, 3
- End-stage renal disease occurs in 1-5% of patients 2
- Average duration of disease is 4 weeks, with most cases being self-limited 3
- Regular follow-up with urinalysis is essential to monitor for development or progression of renal disease 3
- Monitor blood pressure as persistent hypertension can be a long-term complication 3
Treatment Algorithm
Mild disease (skin and joint involvement only):
- Supportive care
- Acetaminophen for pain relief 1
Moderate disease (with abdominal symptoms):
- Supportive care
- Consider prednisone 1-2 mg/kg daily for two weeks 2
Renal involvement:
Severe life-threatening disease:
- High-dose steroids
- Consider methylprednisolone pulse therapy, immunosuppressants, plasma exchange, or IVIG 4