Management of Henoch-Schönlein Purpura
For most children with HSP, supportive care alone is sufficient as the disease spontaneously resolves in 94% of children and 89% of adults, with treatment focused on symptom control and monitoring for renal complications. 1, 2
Initial Diagnostic Confirmation
- Diagnose HSP clinically when palpable purpura is present plus at least one of the following: renal involvement (hematuria and/or proteinuria), arthralgia/arthritis, or abdominal pain 1
- Perform urinalysis with microscopy at presentation to assess for proteinuria, red blood cell casts, and dysmorphic red blood cells indicating glomerular involvement 1
- Obtain basic metabolic panel (BUN, serum creatinine) and complete blood count with platelets to assess renal function and rule out thrombocytopenia 1
- Measure blood pressure as hypertension indicates more severe renal involvement 1
Symptomatic Management
Joint and Cutaneous Symptoms
- Use acetaminophen (paracetamol) as first-line analgesic, avoiding NSAIDs like ketorolac (Toradol) which can cause acute kidney injury in patients with renal impairment 1
- Consider oral prednisone 1-2 mg/kg daily for two weeks for severe joint pain and widespread cutaneous symptoms 1, 2
- For persistent purpura and pain, colchicine 1 mg/day for at least six months may be beneficial 1
Gastrointestinal Symptoms
- Oral prednisone 1-2 mg/kg daily for two weeks is recommended for severe abdominal pain and gastrointestinal hemorrhage 2, 3
- A low-antigen-content (LAC) diet strictly followed for 4-8 weeks may improve symptoms and can be used in conjunction with other treatments 1
- Monitor for complications including intussusception and gastrointestinal bleeding 4
Renal Disease Management Algorithm
Initial Monitoring (All Patients)
- Monitor all HSP patients for six months with regular urinalysis 5
- A normal urinalysis on day 7 has 97% negative predictive value for normal renal outcome 5
- Prioritize intensive monitoring for patients with abnormal urine findings on day 7 5
Persistent Proteinuria
- For persistent proteinuria, initiate ACE inhibitors or ARBs as first-line therapy 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, add a 6-month course of corticosteroid therapy 1
Severe Nephritis
- For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treat with steroids and cyclophosphamide 1, 4
- Alternative immunosuppressants include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid when cyclophosphamide is contraindicated due to side effects 1
Adult Patients
- Apply the same treatment approach as in children for adults with HSP nephritis 1
Important Caveats
- Do not use prophylactic corticosteroids to prevent HSP nephritis - strong evidence shows this is not effective 1
- Older patients are at higher risk for renal complications requiring specialist referral 5
- Long-term prognosis depends entirely on severity of renal involvement, with end-stage renal disease occurring in 1-5% of patients 2
- Adolescents may have more severe presentations requiring close monitoring for gastrointestinal, cardiac, renal, and neurological complications 4
- Renal ultrasound is the preferred imaging modality if renal biopsy is being considered for severe nephritis 1