What is the initial management for a patient with Henoch-Schönlein Purpura (HSP)?

Initial Management of Henoch-Schönlein Purpura (HSP)

The initial management of Henoch-Schönlein Purpura should focus on supportive care, with careful monitoring for organ system involvement, particularly renal manifestations. 1

Diagnosis and Initial Assessment

• HSP diagnosis is based on finding palpable purpura plus at least one of the following: renal involvement (hematuria/proteinuria), abdominal pain, or arthritis 1
• Most patients (>90%) have an antecedent upper respiratory infection 2
• All patients develop purpuric rash, 75% develop arthritis, 60-65% develop abdominal pain, and 40-50% develop renal disease 2

Initial Management Approach

Supportive Care

• HSP is primarily managed with supportive care as it spontaneously resolves in 94% of children and 89% of adults 2
• Acetaminophen (paracetamol) is recommended as a first-line analgesic option for pain management 1
• NSAIDs like ketorolac (Toradol) should be avoided due to risk of acute kidney injury, especially with pre-existing renal impairment 1

Monitoring Requirements

• Regular monitoring of renal function through urinalysis and blood pressure measurements is essential 1
• Patients should be monitored for gastrointestinal complications, which can include bleeding and intussusception 3
• Follow-up should include assessment for potential cardiac and neurological complications, though these are rare 3

Management of Specific Manifestations

Skin and Joint Symptoms

• Supportive care with rest and pain management is typically sufficient 2
• Dapsone may have beneficial effects on cutaneous and articular manifestations in adults with chronic forms 4

Gastrointestinal Symptoms

• For severe abdominal pain that is refractory to supportive measures, corticosteroids may be considered 4
• Oral prednisone at 1-2 mg/kg daily for two weeks has been used to treat abdominal and joint symptoms 2

Renal Involvement

• For HSP nephritis with persistent proteinuria, ACE inhibitors or ARBs are recommended 1
• For persistent 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
• Prophylactic use of corticosteroids is not recommended to prevent HSP nephritis 1
• For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treatment with steroids and cyclophosphamide is recommended 1

Special Considerations

• The presentation of HSP can be more severe in adolescents and adults compared to younger children 3
• Adults with HSP are more likely to experience complications than children 2
• Long-term prognosis depends primarily on the severity of renal involvement, with end-stage renal disease occurring in 1-5% of patients 2
• For severe, life-threatening forms or when renal function is compromised, methylprednisolone pulse therapy, immunosuppressive drugs, plasma exchange, or polyclonal immunoglobulin therapy may be considered 4

Common Pitfalls and Caveats

• Failure to monitor for renal involvement, which is the primary determinant of long-term prognosis 2
• Inappropriate use of NSAIDs, which can worsen renal function 1
• Overlooking potential gastrointestinal complications, which can be severe 5
• Assuming all cases will resolve spontaneously without monitoring, especially in adults who have worse outcomes than children 4