Abstract

Primary IgA nephropathy is a common glomerular disease, heterogeneous in its clinical presentation. Historically considered to assume a benign course, we actually know that up to 40% of the cases progress to end stage renal disease at 20-30 years. Clinical and basic research has now allowed to understand the pathophysiology of this disease, to predict its course and to treat progressive forms more aggressively. This article summarizes classical treatments and recent therapeutic additions (sparsentan and targeted-release budenoside), which demonstrated remarkable efficacy. Complement inhibitors and B cells or plasma cells inhibitors to target aberrant IgA production have already emerged as new potential treatments. Collectively these advances may open a new therapeutic era in the management of this disease.

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