Therapy and prognosis of ANCA-associated vasculitis from the clinical nephrologist's perspective

Abstract

This paper reviews the recently published scientific information regarding ANCA-associated vasculitis (AAV), aiming to highlight the most important data from the clinical nephrologists' perspective. The classification, pathomechanism, recent achievements of the treatment, short-term and long-term outcomes of the disease, and the difficulties nephrologists face when taking care for patients with AAV are summarized. There has been significant progress in the understanding of the genetic and pathologic background of the disease in the last years, and results of histological studies guide us to predict long-term renal function. Findings of several multicentered trials with reasonable number of participants provide comparison of the efficacy and safety of different remission induction and maintenance therapies, and evaluate recently introduced immunosuppressive agents. Although the clinical outcome of patients with AAV has improved significantly since modern immunosuppressive drugs are available, the treatment-related complications still contribute to the morbidity and mortality. To improve the survival and quality of life of patients with AAV further, knowledge of the predictors of relapse, end-stage kidney disease, and mortality, also prevention of infections and other treatment-related adverse events are important. The eligibility for renal transplantation and the option for successful pregnancies for young women are also important factors which influence the patients' quality of life. In order to provide favorable outcome, the clinicians need to establish personalized treatment strategies to optimize the intensity and minimize the toxicity of the immunosuppressive therapy.

Keywords: ANCA-associated vasculitis; Clinical trials; Immunosuppressive therapy; Outcome; Rapidly progressive glomerulonephritis.