Henoch-Schönlein Purpura (IgA Vasculitis): Evaluation and Management
Diagnostic Approach
The diagnosis is made 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
Essential Initial Workup
- Urinalysis with microscopy at presentation and at every follow-up visit to detect proteinuria, red blood cell casts, and dysmorphic red blood cells indicating glomerular involvement 1
- Basic metabolic panel including BUN, serum creatinine to assess renal function 1
- Complete blood count with platelets to confirm non-thrombocytopenic purpura 1
- Blood pressure measurement as hypertension indicates more severe renal involvement 1
- Renal biopsy is not routinely required but should be considered for severe nephritis with nephrotic-range proteinuria or rapidly deteriorating kidney function 2
Monitoring Protocol
- Monitor for at least 6 months after initial presentation, as the majority of children who develop nephritis will do so within 3 months 2, 1
- Perform urinalysis at every clinical visit to detect renal relapse early 1
Management Strategy
Supportive Care for Uncomplicated Disease
Most cases are self-limited with an average duration of 4 weeks and require only supportive measures. 3
- Adequate hydration and rest 3
- Acetaminophen (paracetamol) is the preferred analgesic for joint pain and mild symptoms 1
- Avoid NSAIDs (including ketorolac/Toradol) as they can cause acute kidney injury, especially with pre-existing renal impairment 1
Gastrointestinal Manifestations
- Oral prednisone 1-2 mg/kg/day for 2 weeks may be considered for severe gastrointestinal pain and gastrointestinal hemorrhage 3
- Hospital admission is warranted for clinically significant bleeding or severe abdominal pain 1
Renal Involvement Management
This is the most critical aspect, as renal involvement determines long-term morbidity and mortality. 3
Mild Proteinuria (0.5-1 g/day per 1.73 m²)
- Start ACE inhibitor or ARB therapy for persistent proteinuria despite supportive care 2, 1
- Target proteinuria to <1 g/day/1.73 m² rather than complete normalization 1
- Advise dietary sodium restriction to <2 g per day 1
Moderate to Severe Proteinuria (>1 g/day per 1.73 m²)
- Continue ACE inhibitor or ARB for 3-6 months 2, 1
- If proteinuria persists >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 (oral prednisone/prednisolone or pulsed intravenous methylprednisolone) 2, 1
Crescentic HSP with Nephrotic Syndrome or Rapidly Deteriorating Kidney Function
- Treat with high-dose intravenous methylprednisolone plus cyclophosphamide, following the same protocol as crescentic IgA nephropathy and ANCA-associated vasculitis 2, 1
- This represents life-threatening disease requiring aggressive immunosuppression 2
Critical Management Principles
What NOT to Do
Do not use corticosteroids prophylactically at disease onset to prevent nephritis. Moderate-quality evidence (Grade 1B) demonstrates no benefit in preventing nephritis or reducing the risk of severe persistent nephritis, while exposing patients to unnecessary steroid toxicity. 2, 1, 4
Common Pitfalls to Avoid
- Do not start corticosteroids too early for mild proteinuria without an adequate trial of ACE inhibitor/ARB therapy (3-6 months), as this increases side effects without proven benefit 1
- Do not attempt to normalize proteinuria to <0.5 g/day/1.73 m², which increases medication side effects without proven benefit 1
- Do not fail to perform urinalysis at every clinical visit, as this leads to delayed detection of renal relapse 1
Special Populations
Adults with HSP
- Treat adults using the same approach as children, though adults have worse prognosis and higher risk of progression to end-stage renal disease 2, 1
- Perform age- and sex-appropriate screening for secondary causes and occult malignancy in adult patients 2, 1