IgA Vasculitis in Adults: a Rare yet Challenging Disease

IgA vasculitis (IgAV) is a rare and poorly understood systemic vasculitis in adults. Its diagnosis and treatment remain a challenge. Herein, we review the clinical manifestations, diagnosis, management, and prognosis of IgAV in adults.

The clinical course of IgAV in adults appears to be different from pediatric IgAV, especially due to its higher risk of evolving into end-stage renal disease. Rising awareness and interest in adult-onset IgA vasculitis has resulted in recent increasing number of publications on different treatment experiences. However, there is still controversy over the role of glucocorticoid (GC) and different immunosuppressive therapies such as cyclophosphamide, rituximab, and mychophenolate mofetil for more severe IgAV. Data regarding potential benefits of targeting the mucosal immune system, toll-like receptors, complements, and tyrosine kinase inhibitors in the treatment of IgA nephropathy are emerging. High quality evidence or guidelines in the treatment of severe IgAV are lacking and there is still a great need for controlled trials.