Immunosuppressive management of ANCA-associated glomerulonephritis in children

Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (AAGN) is a rare phenomenon in children and is associated with a significant risk of kidney failure. Over the last decade, there have been updates to clinical guidelines for the immunosuppressive management of this condition, but these continue to be based predominantly on clinical trial data in adults. Recent clinical trials have resulted in increasing use of rituximab for both maintenance and induction treatment, with renewed focus on the role of plasma exchange as part of induction treatment which remains controversial. Newer complement-targeting agents may provide an effective but more targeted and less toxic treatment strategy. This review focuses on immunosuppressive strategies for induction and maintenance treatment, considering recommendations from recent clinical guidelines and how these may be adapted for children and young people with AAGN. Graphical Abstract A higher resolution version of the Graphical Abstract is available as Supplementary information.