Old and New Treatment Options in IgA Nephropathy and Henoch Schönlein Purpura Nephritis/IgA Vasculitis in Children

Purpose of review: Henoch-Schönlein purpura nephritis (HSPN), known currently as nephritis associated with IgA vasculitis (IgAVN), and IgA nephropathy (IgAN) share pathogenetic mechanisms and almost indistinguishable histology, but have peculiar clinical behavior and outcome. Recent classifications, clinical trials, and discussions lead to critical revision of old and new treatments. Recent findings: In recent years, the histological classification of IgAN Oxford-MEST allowed the identification of histological and clinical risk factors for progression of IgAN and encouraged new studies to explore the value of different treatments, allowing a more standardized classification of enrolled patients. For HSPN, large clinical studies are lacking, and the old histological classifications available do not allow such a precise standardization. However, new efforts are made to follow the IgAN pipeline. Personalized therapies are being forecast, tailored according to histological and clinical risk factors implemented by more innovative biomarkers. The aim is to efficaciously treat both diseases in the earlier phases. The inflammatory process can be completely extinguished if treated adequately without delay, with a high percentage of permanent complete remission in children. Summary: The state-of-the-art knowledge of the elements useful to choose the appropriate treatment in HSPN/IgAVN and IgAN in children is reviewed. © 2019, Springer Nature Switzerland AG.