[PROGRESS IN THE PATHOGENESIS, DIAGNOSIS AND TREATMENT OF SYSTEMIC ANCA-ASSOCIATED VASCULITIS]

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic diseases characterized by necrotizing inflammation of small and medium size vessels, without immunologic deposits, and with the presence of specific serum antibodies. Recent genetic and cohort studies should improve the comprehension of the pathogenesis of AAV, the stratification of patients into homogenous groups, and lead to therapeutic implications. Immunosuppressive therapy of AAV includes two major periods: induction of remission and maintenance therapy. Therapy should be chosen individually, not only depending on the stage and severity of the disease, but also on genetic and some prognostic factors. Previous randomized trials and clinical observations show some possible limitations of treatment with cyclophosphamide and steroids. Rytuximab seems to be a good alternative in those patients in induction therapy as well as in maintenance therapy.