Introduction: Antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is a group of autoimmune diseases characterized by the necrotizing inflammation of small vessels and associated with the antineutrophil cytoplasmic antibodies. Treatment of AAV can be divided into the induction phase aimed at achieving remission of the disease and the maintenance phase aimed at prevention of relapses. Long-term outcome of AAV dramatically improved with the introduction of cyclophosphamide. Recent clinical studies resulted in the reduction of the cumulative dose of cyclophosphamide and introduction of new treatment options, namely B-cell-depleting antibody rituximab, into both induction and maintenance treatment. This paper aims to evaluate the current role of the conventional induction and maintenance treatment in view of the gradually increasing use of rituximab.
Areas covered: This paper provides an overview of the main clinical studies in induction and maintenance treatment of adult patients with AAV, treatment of relapses of AAV and shortly comments also on the treatment of refractory AAV, treatment of different subgroups of AAV (based on the age, renal function, clinical presentation and type of autoantibody), long-term outcome of patients with AAV, adverse events of treatment and treatment of end-stage renal disease in AAV.
Expert opinion: Our analysis demonstrates that although the introduction of rituximab modified the approach to both the induction and maintenance treatment of AAV, more conventional induction and maintenance treatment with standard immunosuppressive drugs still retains its importance as we need more data on long-term efficacy and safety of biologic treatment, and also its cost-effectiveness still remains an open issue.
Keywords: antineutrophil cytoplasmic antibodies; antineutrophil cytoplasmic antibodies-associated vasculitis; azathioprine; clinical trial; cyclophosphamide.