What is the initial management for a patient with Henoch Schonlein Purpura (HSP)?

Management of Henoch-Schönlein Purpura

For most patients with HSP, supportive care with monitoring is the primary intervention, as the disease spontaneously resolves in 94% of children and 89% of adults, but aggressive treatment with corticosteroids and immunosuppressants is reserved for severe renal involvement or refractory symptoms. 1, 2

Initial Assessment and Diagnosis

Clinical diagnosis can be made when palpable purpura is present plus at least one of the following: renal involvement (hematuria/proteinuria), arthralgia/arthritis, or abdominal pain 1

Essential Initial Workup

• Urinalysis with microscopy to assess for glomerulonephritis, looking specifically for proteinuria, red blood cell casts, and dysmorphic red blood cells 1
• Basic metabolic panel including BUN, serum creatinine, and complete blood count with platelets to assess renal function and rule out thrombocytopenia 1
• Blood pressure measurement as hypertension indicates more severe renal involvement 1
• Renal ultrasound if severe nephritis is suspected or renal biopsy is being considered 1

Supportive Care (First-Line for Uncomplicated Cases)

Most patients require only supportive treatment as HSP is self-limiting in the majority of cases 2

Pain Management

• Acetaminophen (paracetamol) is the recommended first-line analgesic 1
• Avoid NSAIDs (including ketorolac/Toradol) as they can cause acute kidney injury, especially in patients with pre-existing renal impairment 1

Dietary Considerations

• Low-antigen-content (LAC) diet may improve symptoms and laboratory abnormalities when strictly followed for 4-8 weeks, and can be considered as supportive treatment in all symptomatic patients 1

Treatment of Specific Manifestations

Joint Pain and Cutaneous Symptoms

• Oral prednisone 1-2 mg/kg daily for two weeks may be beneficial for acute, widespread flares, particularly when pain control is needed 1, 2
• Colchicine 1 mg/day may be considered for persistent purpura and pain, with treatment for at least six months 1

Abdominal Pain

• Oral prednisone 1-2 mg/kg daily for two weeks has been shown to reduce the mean time to resolution of abdominal pain 2
• Corticosteroids may be useful for refractory abdominal pain 3

Renal Disease Management (Critical for Long-Term Outcomes)

Renal disease is the most likely complication to result in long-term morbidity, occurring in 40-50% of patients, with end-stage renal disease developing in 1-5% of children and up to 20% of adults 2, 4

Mild to Moderate Proteinuria

• Start ACE inhibitor or ARB therapy for persistent proteinuria, targeting proteinuria to <1 g/day/1.73 m² 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

Severe Renal Involvement

• For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treat with high-dose intravenous methylprednisolone plus cyclophosphamide 1, 3
• Alternative immunosuppressants when needed include steroids combined with azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid 1

Adults with HSP Nephritis

• Treat adults using the same approach as children, though adults have worse prognosis and higher risk of progression to end-stage renal disease 1, 4

Critical Pitfalls to Avoid

• Do NOT use corticosteroids prophylactically at HSP onset to prevent nephritis, as moderate quality evidence shows no benefit in preventing nephritis or reducing risk of severe persistent nephritis 1
• Do NOT start corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy 1
• Do NOT attempt to normalize proteinuria to <0.5 g/day/1.73 m², which increases side effects without proven benefit 1
• Avoid NSAIDs in patients with any degree of renal involvement 1

Monitoring Protocol

All patients require at least 6 months of follow-up with regular monitoring 5, 6

• Regular urine testing for proteinuria and hematuria 1, 5
• Blood pressure measurements at each visit 1
• A normal urinalysis on day 7 has a 97% negative predictive value in predicting a normal renal outcome 6
• Older patients are at higher risk of requiring renal referral and should be monitored more intensively 6

Special Population: Pregnancy

• Women with a history of HSP during childhood are at increased risk of complications (proteinuria and hypertension) during pregnancy and should be monitored closely 5