Highlights • Rapid progressive glomerulonephritis is a leading cause for end-stage renal disease. • IL-17 producing T cells were identified in human nephritic kidneys and in experimental models of crescentic glomerulonephritis. • IL-17 signaling on residential cells contributes to inflammatory tissue damage in experimental glomerulonephritis. • IL-17C promotes the TH17 cell immune response and therefore enhances renal disease pathology. • IL-17 signaling is a potential therapeutic target for rapid progressive glomerulonephritis. Abstract Immune-mediated glomerular diseases (glomerulonephritis) encompass a heterogeneous collection of diseases that cause inflammation within the glomerulus and other renal compartments with significant morbidity and mortality. In general, CD4+ T cells orchestrate the immune response and play a unique role in autoimmune and chronic inflammatory diseases. In particular, the characterization of a distinct, IL-17 cytokines producing CD4+ T cell subset named T H 17 cells has significantly advanced the current understanding of the pathogenic mechanisms of organ-specific immunity. Our group and others have shown that the recruitment of T H 17 cells to the inflamed kidney drives renal tissue injury in experimental and possibly human crescentic glomerulonephritis (GN), but much remains to be understood about the biological functions, regulation, and signaling pathways of the T H 17/IL-17 axis leading to organ damage. Here we review our current knowledge about the mechanisms and functions of IL-17 signaling in renal autoimmune diseases, with a special focus on experimental and human crescentic GN. [ABSTRACT FROM AUTHOR]