From the Research
IgA vasculitis, also known as Henoch-Schönlein purpura, is a systemic vasculitis that typically presents with palpable purpura, arthralgia/arthritis, abdominal pain, and renal involvement, and its treatment should be guided by the severity of symptoms and the presence of renal involvement, with corticosteroids being the first-line therapy for severe manifestations, as reported in the most recent study 1.
Clinical Manifestations
The clinical manifestations of IgA vasculitis include:
- Palpable purpura, which appears predominantly on the lower extremities and buttocks
- Arthralgia or arthritis, which usually affects the knees and ankles with swelling and pain
- Abdominal pain, which can include colicky abdominal pain, vomiting, and occasionally gastrointestinal bleeding
- Renal involvement, which ranges from microscopic hematuria to nephrotic syndrome and occurs in 20-60% of patients These manifestations are caused by the deposition of IgA immune complexes in small vessels, triggered by various factors including infections, medications, or allergic reactions, as described in 2.
Treatment Options
Treatment options for IgA vasculitis depend on the severity of symptoms and the presence of renal involvement. For mild cases, treatment is primarily supportive, including:
- Adequate hydration
- Pain management with acetaminophen or NSAIDs (avoiding NSAIDs if significant renal involvement exists) For severe symptoms, corticosteroids like prednisone at 1-2 mg/kg/day for 1-2 weeks with tapering may be used, particularly for significant gastrointestinal or joint symptoms, as recommended in 1. Severe renal involvement might require higher-dose methylprednisolone (30 mg/kg/day for 3 days) followed by oral prednisone. In cases with rapidly progressive glomerulonephritis or nephrotic syndrome, immunosuppressants such as cyclophosphamide, azathioprine, or mycophenolate mofetil may be added.
Prognosis and Monitoring
Most cases of IgA vasculitis resolve within 4-6 weeks, but approximately one-third of patients may experience recurrences, and long-term monitoring of renal function is essential for those with kidney involvement, as noted in 3. The disease can lead to chronic renal disease, especially in adults, and the long-term prognosis depends on the severity of nephritis, as reported in 2.