Disease models of rapidly progressive glomerulonephritis

Rapidly progressive glomerulonephritis is a life-threatening clinical syndrome that accounts for a disproportionate number of patients requiring renal replacement therapy. The association with organ-specific and systemic autoimmunity is longstanding, but treatment with non-specific cytotoxic immunosuppression, first introduced in the 1970s, is still limited by its efficacy and toxicity. There are now accurate and reproducible rodent models of human diseases and investigation of these models has clarified many of the underlying mechanisms and unveiled targets for therapeutic manipulation that are being explored clinically. It is hoped that future work will lead to more effective, less toxic treatments for patients with renal and other autoimmune conditions.