Treatment of Henoch-Schönlein Purpura (HSP) in Pediatric Patients
Most pediatric HSP cases require only supportive care with NSAIDs for pain, as the disease is self-limited in the majority of patients, and corticosteroids should NOT be used prophylactically to prevent renal involvement. 1
Initial Assessment and Risk Stratification
- Measure blood pressure, serum creatinine, and BUN in all adolescent patients with HSP, as they are at significantly higher risk for severe renal involvement, persistent nephritis, and progression to end-stage renal disease (1-5% overall) compared to younger children 1
- Obtain urinalysis to screen for hematuria and proteinuria at presentation 2, 3
- The diagnosis requires palpable purpura plus at least one of: diffuse abdominal pain, arthritis/arthralgia, renal involvement (hematuria/proteinuria), or biopsy showing predominant IgA deposition 2
Treatment Algorithm by Clinical Presentation
Uncomplicated HSP (Purpura ± Mild Joint Pain)
- Provide supportive care only with NSAIDs for symptomatic pain relief 2
- Do NOT initiate corticosteroids, as moderate-quality evidence demonstrates they do not prevent renal involvement or decrease risk of severe persistent nephritis (Grade 1B recommendation) 1
- The average disease duration is 4 weeks, and most cases resolve spontaneously 2
Severe Gastrointestinal Involvement (Severe Abdominal Pain or GI Bleeding)
- Consider oral corticosteroids (prednisone 1-2 mg/kg/day) for severe gastrointestinal pain and gastrointestinal hemorrhage to hasten symptom resolution 4, 2
- Corticosteroids may shorten the duration of arthritis and abdominal pain but do NOT prevent recurrences 4
- If poor response to steroids with recurrent or persistent severe GI symptoms, consider mycophenolate mofetil as an immunosuppressive alternative, which has shown effectiveness in case reports of steroid-refractory gastrointestinal HSP 5
Renal Involvement - Mild (Microscopic Hematuria ± Mild Proteinuria)
- Initiate ACE inhibitor or ARB for persistent significant proteinuria, targeting reduction to <1 g/day/1.73 m² 1
- Continue ACE inhibitor/ARB for adequate trial of 8-12 weeks before escalating therapy 1
- Monitor urine for proteinuria and hematuria, plus blood pressure, for at least 6 months after HSP onset 3
Renal Involvement - Severe (Nephrotic-Range Proteinuria or Declining Renal Function)
- Perform renal biopsy immediately if the patient presents with decreased renal function, severe nephrotic syndrome, or nephritic syndrome at initial presentation 1
- Start corticosteroid therapy only if nephrotic-range proteinuria persists after adequate trial (8-12 weeks) of ACE inhibitor/ARB 1
- For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, administer high-dose intravenous methylprednisolone immediately 1, 4
- Add cyclophosphamide as the only immunosuppressive agent with RCT evidence for crescentic HSP nephritis in children 1, 4
Critical Pitfalls to Avoid
- Do NOT give prophylactic corticosteroids at HSP onset to "prevent" nephritis, as this is ineffective and exposes patients to unnecessary steroid side effects 1
- Do NOT delay renal biopsy in patients with decreased renal function at presentation, as crescentic disease requires immediate aggressive therapy 1
- Do NOT use corticosteroids in usual doses for established nephritis, as they have no effect on established renal disease 4
Follow-Up and Long-Term Monitoring
- Follow all HSP patients for at least 6 months with regular urine testing for proteinuria and hematuria plus blood pressure measurement 3
- One-third of patients will have one or more recurrences of symptoms, particularly during the first year 4
- Counsel female patients that they are at increased risk of complications (proteinuria and hypertension) during future pregnancies and require close monitoring 3
- Long-term prognosis is directly dependent on the severity of renal involvement, with renal disease being the most important prognostic factor determining morbidity and mortality 4, 2