From the Guidelines
IgA vasculitis typically develops in children between 4-7 years of age. The condition is characterized by a classic tetrad of symptoms, including palpable purpura, arthralgia or arthritis, abdominal pain, and renal involvement 1. The majority of children who will develop nephritis will do so within 3 months of presentation, and management is primarily supportive for most cases.
Key Considerations
- The condition often follows an upper respiratory infection
- Management includes adequate hydration, rest, and pain control with acetaminophen or NSAIDs for joint pain and swelling
- Severe abdominal pain may require hospitalization for monitoring and pain management
- For patients with significant renal involvement, corticosteroids are recommended, typically oral prednisone at 1-2mg/kg/day for 1-2 weeks followed by a taper over 2-4 weeks 1
Treatment Approach
- Oral prednisone/prednisolone or pulsed intravenous methylprednisolone should be used in children with mild or moderate IgAVN
- Children with IgAVN and nephrotic syndrome and/or rapidly deteriorating kidney function are treated in the same way as rapidly progressive IgAN 1
Prognosis
- Most children (>90%) with IgA vasculitis recover completely within 4-6 weeks
- Approximately 30% may experience recurrences The pathophysiology involves IgA immune complex deposition in small vessel walls, triggering inflammation and vasculitis, though the exact trigger remains unclear in most cases.
From the Research
Typical Presentation of IgA Vasculitis
- IgA vasculitis (IgAV) is the most common form of childhood vasculitis, which can occur in any age and peaks around 4-6 years old 2
- The diagnosis is made clinically, and 95% of patients will present with a rash, together with any from a triad of other systems: gastrointestinal, musculoskeletal, and renal 2
- Clinical features and prognosis of pediatric IgAV vary with onset age, with older children being more likely to have renal involvement and younger children being more likely to have joint involvement 3
Age of Onset
- The onset age of IgAV is typically around 4-6 years old, with a median onset age of 6.10 years old 2, 3
- Pediatric IgAV with different onset ages are associated with distinct clinical manifestations and outcomes, with the risk of developing corticosteroid dependence, refractory disease, and renal involvement increasing with onset age 3
Management
- Treatment may be required during the acute phase for gastrointestinal involvement and renal involvement, with oral corticosteroids recommended for milder disease and intravenous corticosteroids with cyclophosphamide recommended for severe disease 2, 4
- Angiotensin system inhibitors can be used as adjunctive treatment for persisting proteinuria, and immunosuppressant agents may be necessary for frequently relapsing disease 2
- International consensus guidelines outline the indications for renal biopsy and the use of medications to prevent subsequent renal inflammation 2, 4