How is Iga Vasculitis treated?
The disease is generally self-limited, lasting from 6 to 16 weeks. For mild cases, supportive treatment alone may be adequate. Arthritis responds to nonsteroidal antiinflammatory drugs (NSAIDs). Systemic glucocorticoids (prednisone 1 mg/kg per day × 2 weeks with tapering off over 2 weeks) may be used in patients with GI involvement or bleeding. Progressive renal disease can be difficult to treat, and data in both pediatric and adult populations is limited. Aggressive treatment with high-dose intravenous glucocorticoid pulses followed by maintenance oral steroids should be considered in patients with poor prognostic factors of proteinuria >1 g/day, nephrotic syndrome, and/or crescentic glomerulonephritis (>50% crescents). Failure to respond after 2 to 3 months of corticosteroid therapy may indicate the need for additional medication such as azathioprine, cyclophosphamide, or rituximab (data is limited in this regard and some clinicians may elect to start immunosuppression immediately in high-risk populations). Given the potential toxicity of immunosuppressive medications and paucity of data regarding their effectiveness, some experts recommend initial treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor blockers for adult patients presenting with proteinuria (including nephrotic range proteinuria) prior to consideration of immunosuppression. However, the decision to withhold immunosuppression in this setting is controversial. Of importance, ANCA-associated vasculitis, which is likely to respond to rituximab, should be ruled out.
