Management and Treatment of Henoch-Schönlein Purpura
For most patients with HSP, supportive care is the primary intervention since the disease spontaneously resolves in 94% of children and 89% of adults, with treatment focused on symptom control and monitoring for renal complications. 1, 2
Diagnostic Confirmation
Before initiating treatment, confirm the diagnosis clinically when palpable purpura is present plus at least one of the following 1:
- Renal involvement (hematuria and/or proteinuria)
- Arthralgia or arthritis
- Abdominal pain
Essential baseline investigations include 1:
- Urinalysis with microscopy (looking for proteinuria, red blood cell casts, and dysmorphic red blood cells)
- Basic metabolic panel (BUN, serum creatinine)
- Complete blood count with platelets (to rule out thrombocytopenia)
- Blood pressure measurement (hypertension indicates severe renal involvement)
Symptomatic Management
Joint and Cutaneous Symptoms
For joint pain and widespread cutaneous symptoms, oral prednisone 1-2 mg/kg daily for two weeks is recommended. 1, 2 This approach reduces the mean time to resolution of abdominal pain and decreases the odds of developing persistent renal disease in children 2.
For persistent purpura and pain, colchicine 1 mg/day for at least six months may be considered 1.
Abdominal Pain
Oral prednisone at the same dosing (1-2 mg/kg daily for two weeks) is effective for refractory abdominal pain 2, 3.
Pain Management Considerations
Avoid NSAIDs like ketorolac (Toradol) in HSP patients, especially those with any renal involvement, as these can cause acute kidney injury. 1 Acetaminophen is the safer first-line analgesic option 1.
Renal Disease Management
Monitoring Protocol
A normal urinalysis on day 7 has a 97% negative predictive value for predicting normal renal outcome, but all patients require six-month monitoring regardless. 4
Monitor for 1:
- Persistent hematuria and proteinuria
- Blood pressure elevation
- Declining renal function
Treatment Based on Severity
For persistent proteinuria (any level), initiate ACE inhibitors or ARBs as first-line therapy. 1
For persistent proteinuria >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. 1
For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function, treat with steroids plus cyclophosphamide. 1 Alternative immunosuppressants include azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil, though cyclophosphamide is preferred for severe disease despite side effects 1, 3.
Prophylactic Steroids
Do not use prophylactic corticosteroids to prevent HSP nephritis—this is not recommended based on strong evidence. 1
Special Populations
Adults
The treatment approach for adults with HSP nephritis should be the same as in children, though adults have worse renal prognosis and are more likely to experience complications 1, 2.
Older Children
Older patients are at significantly higher risk of requiring renal referral (median age 12.3 years vs. 6.0 years for those with normal outcomes), warranting more intensive monitoring 4.
Adjunctive Supportive Measures
A low-antigen-content (LAC) diet may improve symptoms and laboratory abnormalities when strictly followed for 4-8 weeks and can be used in conjunction with other treatments like colchicine for chronic or persistent symptoms 1.
Imaging Considerations
Renal ultrasound is the preferred initial imaging modality to assess kidney size and anatomy, particularly if renal biopsy is being considered for severe nephritis 1.
Long-Term Prognosis
End-stage renal disease occurs in 1-5% of patients, with prognosis depending entirely on the severity of renal involvement 2. Up to 20% of children with nephritis treated in specialized centers may require hemodialysis 3.