Henoch-Schönlein Purpura: Hallmark Symptoms and Management
Henoch-Schönlein purpura (HSP) is characterized by palpable purpura, abdominal pain, arthritis/arthralgia, and renal involvement, with management primarily consisting of supportive care and targeted interventions for organ-specific complications, particularly kidney disease. 1
Hallmark Clinical Manifestations
Palpable purpura: Non-thrombocytopenic purpura predominantly on the lower extremities and buttocks is the most characteristic finding, present in virtually all patients with HSP 1
Joint involvement: Arthritis or arthralgia affects primarily weight-bearing joints (knees and ankles), occurring in approximately 75% of cases 1, 2
Gastrointestinal manifestations: Abdominal pain, often colicky in nature, occurs in 50-75% of patients and may be complicated by gastrointestinal bleeding or intussusception 1, 2
Renal involvement: Ranges from microscopic hematuria to severe nephritis with proteinuria, nephrotic syndrome, or acute kidney injury; occurs in 20-60% of patients and represents the most important prognostic factor 1, 3
Neurological manifestations: Less common but can include headaches, behavioral changes, seizures (in 53% of patients with neurologic complications), and rarely more severe CNS vasculitis 4
Diagnostic Criteria
- Diagnosis should be based on finding palpable purpura plus at least one of the following 1:
- Diffuse abdominal pain
- Arthritis or arthralgia
- Renal involvement (hematuria and/or proteinuria)
- Biopsy showing predominant IgA deposition
Management Approach
General Management
Most cases are self-limited with an average duration of 4 weeks, requiring only supportive care 1, 2
Monitoring for complications, particularly renal involvement, is essential as this determines long-term prognosis 1
Specific Organ System Management
Renal Disease Management
For children with HSP nephritis and persistent proteinuria (0.5-1 g/day per 1.73 m²): Treatment with ACE inhibitors or ARBs is recommended 5
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 5
For crescentic HSP with nephrotic syndrome and/or deteriorating kidney function: Treatment with steroids and cyclophosphamide is recommended, similar to the approach for ANCA vasculitis 5
For adults with HSP nephritis: Treatment approach should be the same as in children 5
Prophylactic use of corticosteroids: Not recommended to prevent HSP nephritis (strong evidence) 5
Gastrointestinal Manifestations
For severe gastrointestinal pain and hemorrhage: Oral corticosteroids may be considered 1
For intussusception: Prompt surgical consultation and appropriate intervention 1
Special Considerations
Recurrent episodes: No specific evidence-based recommendations exist for preventing recurrences 3
Adult patients: Treatment approach should mirror that used in children, though adults may have more severe disease course 5, 2
Immunosuppressive therapy options: When needed for severe nephritis, options include steroids combined with azathioprine, cyclosporine, tacrolimus, or mycophenolate mofetil/mycophenolic acid; cyclophosphamide is used less frequently due to side effects 5
Monitoring and Follow-up
Regular monitoring of blood pressure, urinalysis, and kidney function is essential, particularly in the first 6-12 months 1
Long-term complications are rare but include persistent hypertension and end-stage kidney disease 1
For patients with severe nephritis or persistent proteinuria >3 months, ACE inhibitors or ARBs should be considered in addition to corticosteroids to prevent secondary glomerular injury 1
Treatment Efficacy and Limitations
Current evidence does not support universal treatment with corticosteroids for all HSP patients 1, 3
Early steroid treatment does not reduce the incidence or severity of nephropathy in children with HSP 1
Limited evidence exists for the efficacy of various immunosuppressive agents in severe HSP nephritis, with studies showing no clear benefit of cyclophosphamide over supportive care 3