Immune-mediated glomerular diseases are a leading cause for the development of end-stage renal failure in the Western world. Glomerular injury caused by the immune system can be a consequence of systemic diseases, such as ANCA-associated vasculitis or systemic lupus erythematosus (SLE), or it can be due to a kidney restricted disease, such as primary membranous nephropathy and most forms of membranoproliferative glomerulonephritis. These entities differ substantially from one another in their primary localization of glomerular injury, clinical course and manifestations. The clinical symptoms range from asymptomatic hematuria to nephrotic syndrome with massive urinary protein loss or even worse rapidly progressive glomerulonephritis (RPGN) with irreversible renal failure (Figure 1). Fundamental to all forms of immune-mediated glomerular injuries is a disturbed immune reaction, which leads to local pathogenic inflammatory responses within the glomerulus and other compartments of the kidney. This pathophysiological concept is the current rationale for unspecific (and often unsuccessful) immunosuppressive treatment consisting of corticosteroids and cytotoxic agents. A lack of specificity of these therapeutic regimens and the frequently disabling side effects heighten the urgency of developing new and more specific individual therapeutic strategies.