What is new in the therapy of ANCA-associated vasculitides? Take home messages from the 12th workshop on ANCA and systemic vasculitides.

摘要

ANCA-associated systemic vasculitides (AASV) were previously fatal diseases, in which long-term survival is now achieved with cyclophosphamide (CYC) and prednisolone. As this standard treatment has shown considerable long-term morbidity and mortality and as more sensitive diagnostic procedures allow the earlier diagnosis of these diseases, nowadays, stage adapted treatment regimens that reduce the exposure to CYC are required. There is consensus that at present CYC remains the drug of choice in patients with generalized vasculitis for the induction of a remission period comprising 3-6 months. Whether pulse CYC is to be preferred over daily oral CYC is currently assessed in a RCT. There are efforts to further minimize the cumulative CYC dose for remission induction in elderly people, because the mortality is highest, and by adding monoclonal anti-B-cell antibodies. Adding Etanercept to the conventional induction regimen has not proven beneficial in a US RCT. For maintenance of remission a switch from CYC to azathioprine has proven to be safe. Methotrexate for this indication has been found to be comparable to azathioprine in one trial, but was associated with more relapses than leflunomide in another. Mycophenolate mofetil is currently studied with 48 months follow-up time. For induction of remission in patients without renal insufficiency and vital organ failure methotrexate at 0.3 mg/kg/week can replace CYC in patients with moderately extended disease and without pronounced granulomatous changes in the respiratory tract. Myfortic will be assessed for a similar indication in the future. Currently, long-term follow-up of the EUVAS patients is also sought.