Minimal change disease articles from across Nature Portfolio

Although B cell-targeting therapies can provide clinical benefits to children with idiopathic nephrotic syndrome (INS), B lymphocyte subsets have not been extensively studied in this disease. Here, using single-cell RNA sequencing, the authors identify an extrafollicular B cell signature in children with INS.

Here, the authors have performed a multi-population GWAS meta-analysis of pediatric steroid sensitive nephrotic syndrome cases to discover 12 loci (4 novel), fine-map HLA, implicate kidney and immune factors, and associate the polygenic risk score with earlier disease onset.

Over the past year, trial data have emerged on therapeutic interventions in IgA nephropathy and lupus nephritis, including the effects of different doses of glucocorticoids and several novel targeted therapies. These data, in combination with the discovery of autoantibodies targeting nephrin in minimal change disease, can inform the management of immune-mediated glomerular diseases.

A new trial provides further evidence that rituximab treatment can reduce the risk of relapse in children with steroid-dependent idiopathic nephrotic syndrome. Addition of a single infusion of rituximab to steroid therapy was associated with a decrease in proteinuria at 3 months and a significant increase in the relapse-free period.

Anti-CD20 therapy is increasingly being used in the treatment of various patterns of nephrotic syndrome in adults and children. However, its use is still based largely on observational studies and expert opinion. Well-designed randomized controlled trials are urgently needed to define the role of this expensive therapy.