[Renal disease in ANCA-associated vasculitis]

Renal disease in ANCA-associated vasculitis (AAV) is characterized by a pauci-immune necrotizing glomerulonephritis. Renal biopsy is essential for diagnosis, treatment strategy and also for the long-term prognosis of AAV. The prospective randomized trials conducted by the EUVAS and GFEV have contributed to define therapeutic guidelines. The current standard of care can be divided into two phases: initial immunosuppression for rapid and effective onset of remission and subsequent maintenance therapy to prevent relapses. Despite these recent therapeutic advances, mortality and morbidity, such as end-stage renal failure, remain high, related to diagnosis delay, drug-related toxicity, refractory disease and propensity of AAV to relapse. Conventional therapies need to be optimized, especially in high-risk patients. Targeting B-cells with rituximab is a new and attractive therapeutic option, but long term benefits and safety are still unknown.