Management of Henoch-Schönlein Purpura (HSP)
The management of Henoch-Schönlein Purpura should focus on supportive care for most patients, with specific interventions for organ involvement, particularly renal disease, which is the main determinant of long-term prognosis. 1
Diagnosis
- 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 common in children 2-10 years old, with peak incidence at 4-7 years 2
- 90% of cases occur in children, though adults tend to have more severe disease and complications 3
General Management Approach
- Supportive care is the primary intervention as HSP spontaneously resolves in 94% of children and 89% of adults 3
- Monitor all patients for at least 6 months with regular urine testing for proteinuria/hematuria and blood pressure measurements 4
- Pain management should prioritize acetaminophen as first-line analgesic rather than NSAIDs (like ketorolac) due to potential renal complications 1
Specific Organ System Management
Skin and Joint Manifestations
- All patients develop purpuric rash, and approximately 75% develop arthritis 3
- For joint pain and cutaneous symptoms, oral prednisone at 1-2 mg/kg daily for two weeks may be beneficial 3
- Colchicine (1 mg/day) may be considered for persistent purpura and pain, with treatment for at least six months 5
Gastrointestinal Manifestations
- Abdominal pain occurs in 60-65% of patients 3
- Oral corticosteroids should be considered for patients with severe gastrointestinal pain and gastrointestinal hemorrhage 2
- A meta-analysis found that corticosteroid use in children reduced the mean time to resolution of abdominal pain 3
Renal Disease Management
- Renal involvement occurs in 40-50% of patients and is the main determinant of long-term prognosis 3
- For persistent proteinuria, ACE inhibitors or ARBs are recommended as first-line therapy 1
- For children with 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
- 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
- End-stage renal disease occurs in 1-5% of patients 3
Severe or Refractory Disease
- For severe nephritis, options include steroids combined with azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil 1
- Cyclophosphamide is used less frequently due to side effects 1
- For very rare life-threatening forms, methylprednisolone pulse therapy, immunosuppressive drugs, plasma exchange, and polyclonal immunoglobulin therapy may be beneficial 6
Dietary Considerations
- A low-antigen-content (LAC) diet may improve symptoms and laboratory abnormalities when strictly followed for 4-8 weeks 5
- LAC diet can be considered as supportive treatment in all symptomatic patients 5
Long-term Follow-up
- Women with a history of childhood HSP require close monitoring during pregnancy due to increased risk of complications such as proteinuria and hypertension 4
- The average duration of the disease is 4 weeks, but long-term complications can include persistent hypertension and end-stage kidney disease 2
Treatment Algorithm
- Initial presentation: Supportive care with hydration and acetaminophen for pain
- For severe abdominal pain or joint symptoms: Consider prednisone 1-2 mg/kg daily for two weeks
- For renal involvement:
- Monitor urine for proteinuria and hematuria
- If persistent proteinuria: Start ACE inhibitor or ARB
- If proteinuria >1g/day persists after ACE/ARB: Add 6-month course of corticosteroids
- If crescentic nephritis or deteriorating function: Steroids plus cyclophosphamide
- For chronic or persistent symptoms: Consider colchicine or LAC diet
- Follow-up: Monitor all patients for at least 6 months with regular urine testing and blood pressure checks