Management of Henoch-Schönlein Purpura (IgA Vasculitis) in Children
For a child presenting with HSP, provide supportive care as the primary approach since most cases are self-limited, reserve corticosteroids only for severe gastrointestinal complications or significant nephritis, and monitor renal function closely for at least 6 months since nephritis determines long-term prognosis. 1
Initial Diagnostic Confirmation
The diagnosis is clinical and requires palpable purpura plus at least one of the following: 1
- Renal involvement (hematuria and/or proteinuria)
- Arthralgia or arthritis
- Abdominal pain
- Biopsy showing predominant IgA deposition
Perform urinalysis with microscopy at every clinical encounter to detect hematuria, proteinuria, red blood cell casts, and dysmorphic red blood cells indicating glomerular involvement. 1 Measure blood pressure at each visit, as hypertension signals more severe renal disease. 1
Obtain basic metabolic panel (BUN, creatinine) and complete blood count with platelets to assess renal function and exclude thrombocytopenia. 1
Management of Extrarenal Manifestations
Cutaneous and Joint Symptoms
Most skin manifestations and arthralgia resolve spontaneously within 4 weeks without specific treatment. 2 For significant joint pain or widespread cutaneous symptoms, oral prednisone 1-2 mg/kg daily for two weeks may provide symptomatic relief. 1
Avoid NSAIDs like ketorolac (Toradol) in HSP patients due to risk of acute kidney injury, especially with any degree of renal involvement. 1 Use acetaminophen as the first-line analgesic instead. 1
Gastrointestinal Complications
For severe abdominal pain or gastrointestinal bleeding, oral corticosteroids (prednisone 1-2 mg/kg/day) are indicated. 2 This represents one of the few clear indications for steroid therapy in HSP. 2
Do not use prophylactic corticosteroids at disease onset to prevent nephritis—moderate quality evidence (Grade 1B) demonstrates no benefit in preventing nephritis or reducing risk of severe persistent nephritis, while exposing patients to unnecessary steroid toxicity. 1
Renal Disease Management: A Tiered Approach
Mild Proteinuria (Persistent but <1 g/day/1.73 m²)
Start ACE inhibitor or ARB therapy for persistent significant proteinuria, even though evidence is extrapolated from IgA nephropathy rather than HSP-specific trials. 1 Target proteinuria to <1 g/day/1.73 m² rather than attempting complete normalization, which increases medication side effects without proven benefit. 1
Moderate Nephritis (Proteinuria >1 g/day/1.73 m² after ACE inhibitor/ARB trial)
If proteinuria persists >1 g/day per 1.73 m² after adequate trial of ACE inhibitor/ARB therapy AND GFR remains >50 ml/min per 1.73 m², add a 6-month course of corticosteroid therapy. 1 Reserve corticosteroids for nephrotic syndrome (proteinuria >3.5 g/day) or nephritic syndrome that has not improved with ACE inhibitor/ARB therapy. 1
Severe/Crescentic Nephritis
For crescentic HSP with nephrotic syndrome and/or rapidly deteriorating kidney function, treat immediately with high-dose intravenous methylprednisolone plus cyclophosphamide, following the same protocol as crescentic IgA nephropathy. 1 This represents true rapidly progressive glomerulonephritis requiring urgent immunosuppression. 3
Alternative immunosuppressive agents for severe nephritis include steroids combined with azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid, though cyclophosphamide is preferred for the most severe cases despite side effect concerns. 1
Critical Monitoring Protocol
Monitor all HSP patients for proteinuria and renal function for a minimum of 6 months, since the majority of children who develop nephritis will do so within 3 months of presentation. 1 Continue urinalysis at every clinical visit throughout this period. 1
Perform renal ultrasound if severe nephritis develops or if renal biopsy is being considered. 1
Common Pitfalls to Avoid
Do not start corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy—this increases side effects without proven benefit. 1
Do not attempt to normalize proteinuria to <0.5 g/day/1.73 m², as this increases side effects without proven benefit. 1
Do not delay urinalysis at follow-up visits—failing to perform urinalysis at every clinical encounter can lead to delayed detection of renal relapse or complications. 1
Adjunctive Supportive Measures
Advise dietary sodium restriction to less than 2 g per day to support renal health. 1 A low-antigen-content diet may improve symptoms when strictly followed for 4-8 weeks and can be considered as supportive treatment in symptomatic patients. 1
Special Considerations for Adults
Adults with HSP should receive the same treatment approach as children, though they have worse prognosis and higher risk of progression to end-stage renal disease. 1 Perform age- and sex-appropriate screening for secondary causes and occult malignancy in adult patients. 1