[New insights in the treatment of ANCA-associated systemic vasculitis]

The prognosis of untreated ANCA-associated systemic vasculitis (AASV) is poor with up to 90% of patients dying within 2 years. The combination of prednisone and cyclophosphamide is now established as the treatment of choice and leads to control of the disease in 80-90% of the patients. Such treatment has turned these acutely fatal diseases into chronic relapsing disorders with accumulating drug-related morbidity in over 50% of the patients, including diabetes, bladder and lympho-proliferative malignancy and infertility. Treatment of AASV can be divided into three phases: therapy for induction of remission, for maintenance of remission and therapy for refractory and relapsing disease. In addition, the treatment must be tailored to the stage and severity of the disease and a new classification of AASV was introduced: localized vasculitis, early systemic vasculitis, generalized vasculitis, severe renal vasculitis and refractory vasculitis. Different randomized clinical trials have been performed with the aim of optimizing the existing therapeutic regimens: some of these have been concluded, others are still ongoing. Newer therapeutic approaches are currently being tested and have involved the use of mycophenolate mofetil, anti tumour necrosis factor (TNF) drugs (anti TNF antibody infliximab and humanized soluble TNF receptor etanercept), monoclonal anti- lymphocyte antibody (anti-CD20). These new alternative therapies could be used in patients with frequent relapses, in patients who fail to achieve remission with standard induction therapy and in those with severe side effects due to cumulative doses of cyclophosphamide.