T helper (Th) cells are an integral part of the host's immune response to eliminate invading pathogens. However, autoimmune or 'autoinflammatory' diseases can develop if Th cell responses are not effectively regulated. Several subsets of Th cells exist, including the Th17 subset that produces interleukin-17A, important in experimental models of organ-specific autoimmune inflammation. Its discovery has explained paradoxical observations in model systems thought to be Th1 mediated but were exacerbated in the absence of interferon-gamma, the prototypic Th1 effector cytokine. Th17 cells express unique transcription factors and secrete a unique pattern of cytokines. Interleukin-17A induces pro-inflammatory cytokines and chemokines and mediates neutrophil recruitment. Th17 cells have a reciprocal relationship with T regulatory cells and can also mediate suppression of Th1 responses. Recent studies also suggest that Th17 cells are not terminally differentiated but can switch into Th1 cells. Th17 cells have themselves been recently shown to induce antigen-specific cell-mediated proliferative glomerulonephritis. There is increasing evidence implicating Th17 cells in anti-glomerular basement membrane disease, lupus nephritis and pauci-immune glomerulonephritis. This review will review the discovery of the Th17 subset, its properties, its relationship with other Th subsets and assess the current evidence implicating Th17 cells in glomerulonephritis.