<p>Immunoglobulin A nephropathy (IgAN) is the most frequent form of glomerulonephritis worldwide and a&#xa0;major cause of kidney failure, especially in young adults. Based on current knowledge, the prognosis of the disease is primarily determined by proteinuria and the annual loss of kidney function. Despite intensive research, no specific biomarkers have so far been identified; however, galactose-deficient IgA (Gd-IgA) is currently being evaluated as a potential follow up parameter in ongoing clinical trials. New findings on the pathophysiology include information on changes in the microbiome that could affect the glycosylation of secreted IgA and its reabsorption into the circulation as well as IgA binding to antigens on mesangial cells; however, the most relevant innovations concern the treatment of IgAN. The update of the IgAN chapter of the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines includes new targets and revised recommendations for treatment strategies. In addition, numerous new publications from clinical studies have been published, reporting successful treatment in patients with IgAN. This dynamic development in IgAN research underlines that the portfolio of innovative treatment approaches and personalized treatment strategies could sustainably improve the prognosis of patients.</p>