What is the management and treatment approach for a patient with Henoch-Schönlein purpura (HSP)?

Management of Henoch-Schönlein Purpura

For most patients with HSP, supportive care alone is sufficient, with corticosteroids reserved only for severe gastrointestinal pain or hemorrhage; prophylactic steroids do not prevent nephritis and should not be used. 1

Initial Diagnostic Workup

When HSP is suspected, confirm the diagnosis clinically by identifying palpable purpura plus at least one of the following: renal involvement (hematuria/proteinuria), arthralgia/arthritis, or abdominal pain. 1, 2 The classic triad of hematuria, purpuric lesions, and ankle pain is specifically diagnostic. 1, 2

Essential baseline testing includes:

• Urinalysis with microscopy looking specifically for proteinuria, red blood cell casts, and dysmorphic RBCs indicating glomerular involvement 1
• Basic metabolic panel (BUN, creatinine) to assess renal function 1
• Complete blood count with platelets to rule out thrombocytopenia 1
• Blood pressure measurement, as hypertension indicates more severe renal involvement 1

General Supportive Management

Provide all patients with:

• Pain control with acetaminophen as first-line analgesic 1
• Dressings for any pus-producing lesions 3
• Patient education materials 3

Avoid NSAIDs (including ketorolac/Toradol) as they can cause acute kidney injury, especially problematic given the high risk of renal involvement in HSP. 1

Gastrointestinal Manifestations

For severe abdominal pain or gastrointestinal hemorrhage, consider oral prednisone 1-2 mg/kg daily for two weeks. 1, 4 However, corticosteroids do not shorten disease duration or prevent recurrences. 5

Do not perform endoscopy acutely if perforation or severe obstruction is suspected, as insufflation significantly increases perforation risk. 6

Renal Disease Management Algorithm

This is the most critical aspect of HSP management, as renal involvement determines long-term morbidity and mortality. 2, 4

Monitoring Protocol

Perform urinalysis at every clinical visit for at least 6 months after presentation. 1, 7 A normal urinalysis on day 7 has a 97% negative predictive value for normal renal outcome, but monitoring must continue. 7

Treatment Based on Severity

For persistent proteinuria (any level):

• Start ACE inhibitor or ARB therapy as first-line treatment 1, 4
• Target proteinuria to <1 g/day/1.73 m² rather than attempting complete normalization 1

For persistent proteinuria >1 g/day/1.73 m² after ACE inhibitor/ARB trial AND GFR >50 ml/min/1.73 m²:

• Add a 6-month course of corticosteroid therapy 1

For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function:

• Treat with high-dose IV methylprednisolone plus cyclophosphamide 1, 8, 5
• This represents severe disease requiring aggressive immunosuppression 8

Alternative immunosuppressive options when cyclophosphamide is contraindicated:

• Azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid 1
• However, evidence for these agents is limited 9

Adults with HSP Nephritis

Treat using the same approach as children, though adults have worse prognosis and higher risk of progression to end-stage renal disease. 1, 2

Critical Pitfalls to Avoid

Do not use prophylactic corticosteroids at HSP onset to prevent nephritis - moderate quality evidence shows no benefit in preventing nephritis or reducing risk of severe persistent nephritis. 1, 4 This is a common error that exposes patients to steroid side effects without benefit.

Do not start corticosteroids too early for mild proteinuria without adequate trial of ACE inhibitor/ARB therapy first. 1

Do not fail to perform urinalysis at every clinical visit - this leads to delayed detection of renal relapse or progression. 1, 2

Do not attempt to normalize proteinuria to <0.5 g/day/1.73 m² as this increases medication side effects without proven benefit. 1

Screening for Comorbidities

Screen all patients for depression, anxiety, and cardiovascular risk factors (diabetes, hypertension, hyperlipidemia, central obesity). 3 If persistent gastrointestinal symptoms occur, refer for inflammatory bowel disease screening. 3

Special Populations

Older patients are at higher risk for requiring renal referral and should be monitored more intensively. 7

Approximately 20-50% of children develop nephritis, which represents the major determinant of long-term morbidity and mortality. 2 Most cases are self-limited with average disease duration of 4 weeks, but one-third will have recurrences. 5

Adjunctive Therapies

For persistent purpura and pain, colchicine 1 mg/day may be considered for at least six months. 1 A low-antigen-content diet strictly followed for 4-8 weeks may improve symptoms when used as supportive treatment. 1