review · Journal of Clinical Medicine
IgA vasculitis is the most frequent systemic vasculitis in children, but it presents more severely in adults, where glomerulonephritis leads to end-stage renal disease in ten to thirty percent of cases. Glucocorticoids remain the standard first-line therapy, particularly for severe adult presentations. Mild manifestations can be managed with colchicine, dapsone, or methotrexate. To minimise steroid-related toxicity, immunomodulatory agents including cyclosporine A, tacrolimus, and mycophenolate mofetil serve as effective steroid-sparing alternatives, while leflunomide requires additional evaluation. Rituximab demonstrates clear efficacy in cutting relapse rates, lowering cumulative steroid burdens, and securing long-term remission across age groups. For life-threatening episodes, immunoglobulins and plasma exchange provide utility. Furthermore, early findings show promise for newer interventions such as targeted budesonide formulations, B-cell inhibitors, complement blockers, and sodium-glucose cotransporter-2 inhibitors.
IgA vasculitis can lead to severe organ damage, particularly in adults who face a significant risk of progressing to end-stage kidney failure. Identifying effective steroid-sparing therapies and advanced biological options is vital for preventing permanent renal injury, minimising the toxic side effects of long-term steroid usage, and establishing clearer management pathways for vulnerable patients.
The findings outline therapeutic applications relevant to pharmaceutical developers, nephrologists, and rheumatologists treating autoimmune renal disorders. Interventions range from established, market-ready medicines such as rituximab and calcineurin inhibitors to clinical-stage candidates such as complement pathway inhibitors, endothelin receptor antagonists, and targeted-release budesonide, which require further clinical research and validation before broader therapeutic adoption.
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<b>Objective:</b> IgA vasculitis (IgAV), previously named as Henoch-Schönlein purpura, is the most frequent systemic vasculitis in children. In adults, IgAV is less common although it is associated with more severe disease. In fact, the frequency of glomerulonephritis (referred to as IgAV nephritis) in adults is higher than in children and tends to present more severely, with around 10-30% of those affected eventually progressing to end-stage renal disease. In this review, we describe the pathophysiology, main clinical features, diagnosis of the disease, and latest clinical data regarding IgAV therapy. <b>Methods:</b> A narrative literature review, primarily based on articles published in PubMed, was conducted. In addition to discussing the main aspects of glucocorticoids and conventional disease-modifying drugs used in the management of IgAV, this review focuses on the latest information reported regarding biologics and potential future therapies. <b>Results:</b> Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. Colchicine, dapsone, and methotrexate can be useful for controlling minor manifestations. Several immunomodulatory agents, such as cyclosporine A, tacrolimus, and mycophenolate mofetil, have shown favorable results as glucocorticoid-sparing agents. Leflunomide has shown promising results but requires further study. The use of 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. Immunoglobulins and plasma exchange therapy can also be useful in difficult and life-threatening situations. Other potential therapies with encouraging results include TRF-budesonide, B-cell-directed therapy, B-cell-depleting agents, sodium-glucose cotransporter-2 inhibitors, endothelin receptor antagonists, and complement pathway inhibitors. <b>Conclusions:</b> Glucocorticoids are the first-line therapy for IgAV, especially in adults with severe manifestations. The role of various immunomodulatory therapies, such as calcineurin inhibitors and mycophenolate mofetil, remains promising, while rituximab reduces the long-term side effects of glucocorticoids and can help achieve disease remission. Other potential therapies with encouraging results require further research.
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DOI: 10.3390/jcm13216621
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