Treatment strategies for ANCA-associated vasculitides

Abstract

Recent data from randomized controlled clinical trials have allowed the development of recommendations for treatment of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides (AAV). A selective literature search was carried out for studies and recommendations for treatment of AAV. In active severe AAV a combination of prednisolone and cyclophosphamide or rituximab leads to a therapeutic response in approximately 90 % of cases. Once remission is attained the administration of azathioprine or methotrexate for 2-4 years is required for maintenance of remission. Relapse occurs in more than 30 % of patients despite maintenance treatment. In cases of persistence or progression of disease activity during standard therapy, referral to an expert center should be considered. Despite improvement in the prognosis in recent years early mortality is increased, particularly due to infections. Stage and activity adapted treatment strategies have improved the outcome of AAV in the past three decades. The elevated early mortality and the risk of relapse show the need for further improvement of current treatment protocols with respect to substance selection, dosage of glucocorticoids and immunosuppressants and the duration of therapy.