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

Henoch-Schönlein Purpura Treatment Protocol

Most patients with HSP require only supportive care, as the disease spontaneously resolves in 94% of children and 89% of adults, with treatment focused on symptom management and monitoring for renal complications rather than disease modification. 1, 2

Initial Assessment and Risk Stratification

Upon diagnosis, immediately perform:

• Urinalysis with microscopy to assess for proteinuria, red blood cell casts, and dysmorphic red blood cells indicating glomerular involvement 1
• Basic metabolic panel including BUN and serum 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

Treatment Algorithm by Disease Severity

Mild Disease (Skin, Joint, Mild Abdominal Symptoms)

Supportive care alone is the primary intervention for patients without significant renal involvement or severe symptoms 2, 3. This includes:

• Rest and hydration
• Acetaminophen for pain control (avoid NSAIDs like ketorolac due to risk of acute kidney injury in patients with potential renal involvement) 1
• No corticosteroids, as current evidence does not support universal treatment with steroids 3

Moderate Disease (Severe Abdominal Pain, Significant Joint Pain)

Oral prednisone 1-2 mg/kg daily for two weeks may be used for severe abdominal pain and joint symptoms 1, 2. A meta-analysis demonstrated that corticosteroids reduce the mean time to resolution of abdominal pain 2. However, this does not prevent renal disease development 3.

Colchicine 1 mg/day for at least six months may be considered for persistent purpura and pain 1.

Renal Disease Management

Mild Renal Involvement (Microscopic Hematuria, Mild Proteinuria)

Start ACE inhibitor or ARB therapy for persistent proteinuria, even though evidence is extrapolated from IgA nephropathy rather than HSP-specific trials 1. Target proteinuria reduction to <1 g/day per 1.73 m² 1.

Do not use prophylactic corticosteroids at HSP onset to prevent nephritis, as moderate quality evidence shows no benefit in preventing nephritis or reducing risk of severe persistent nephritis 1, 4.

Moderate Renal Involvement (Persistent Proteinuria >1 g/day per 1.73 m²)

After 3-6 months of optimized ACE inhibitor/ARB therapy, if proteinuria persists >1 g/day per 1.73 m² and GFR remains >50 ml/min per 1.73 m²:

• Add a 6-month course of corticosteroid therapy 1
• This approach is recommended by the American Journal of Kidney Diseases and Kidney International 1

Severe Renal Involvement (Crescentic HSP, Nephrotic Syndrome, Deteriorating Kidney Function)

Treat with high-dose steroids plus cyclophosphamide for crescentic HSP with nephrotic syndrome and/or deteriorating kidney function 1, 2. This follows the same protocol as crescentic IgA nephropathy 1.

Alternative immunosuppressants when needed include:

• Azathioprine
• Cyclosporine
• Tacrolimus
• Mycophenolate mofetil/mycophenolic acid 1

Note that cyclophosphamide is used less frequently due to side effects 1, and evidence from RCTs has not demonstrated clear benefit 4.

Monitoring Protocol

All patients require follow-up for at least 6 months with regular monitoring including 5:

• Urinalysis for proteinuria and hematuria
• Blood pressure measurements
• Assessment for persistent symptoms

Women with childhood HSP history are at increased risk of proteinuria and hypertension during pregnancy and require close monitoring 5.

Common Pitfalls to Avoid

• Do not start corticosteroids prophylactically at disease onset to prevent nephritis—this has no proven benefit 1, 3
• Do not use NSAIDs for pain control in patients with potential renal involvement due to risk of acute kidney injury 1
• Do not attempt to normalize proteinuria to <0.5 g/day per 1.73 m², as this increases side effects without proven benefit 1
• Do not use antiplatelet agents or heparin routinely, as evidence does not support benefit for preventing persistent kidney disease 4

Special Populations

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. End-stage renal disease occurs in 1-5% of all patients 2.

Supportive dietary measures including a low-antigen-content (LAC) diet may improve symptoms when strictly followed for 4-8 weeks and can be considered as adjunctive treatment 1.