From the Research
IgA vasculitis, also known as Henoch-Schönlein purpura, is a systemic, immune complex-mediated, small-vessel leukocytoclastic vasculitis characterized by nonthrombocytopenic palpable purpura, arthritis, and abdominal pain, with a high spontaneous resolution rate in both children and adults. This condition primarily affects children between ages 2-10 but can also occur in adults, with the disease typically causing a distinctive purplish rash on the lower extremities and buttocks, along with joint pain, abdominal pain, and sometimes kidney involvement 1. The diagnosis is usually made clinically based on the characteristic rash and other symptoms, with diagnostic testing required only to exclude other etiologies of purpura, to identify renal involvement, and, if indicated, to determine its extent with biopsy.
Key Characteristics and Management
- The condition results from IgA immune complexes depositing in small vessel walls, triggering inflammation.
- Most cases resolve on their own within a few weeks without specific treatment, though supportive care with pain relievers like acetaminophen or ibuprofen is often recommended for symptom management.
- In more severe cases, especially those with significant kidney involvement, glucocorticoids are the first-line therapy, especially in adults with severe manifestations 2.
- Other treatments such as colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations, while immunomodulatory agents like cyclosporine A, tacrolimus, and mycophenolate mofetil have shown favorable results as glucocorticoid-sparing agents.
- Rituximab has demonstrated efficacy in reducing relapse frequency, lowering the cumulative glucocorticoid burden, and achieving long-term remission of the disease in children and adults with IgAV 2.
Prognosis and Follow-Up
- While most patients recover completely, about 1-5% may develop long-term kidney problems, making follow-up urinalysis important to monitor for persistent protein or blood in the urine.
- The long-term prognosis depends on the extent of renal involvement, with factors associated with long-term end-stage renal disease (ESRD) including baseline renal function impairment and baseline proteinuria >1 or 1.5 g/day, and on renal biopsy degree of interstitial fibrosis, sclerotic glomeruli, and fibrinoid necrosis 3.
- Six months of follow-up is prudent to assess for disease relapse or remission 1.