Abstract

Treatment of ANCA-associated vasculitides is better codified and new strategies improved diseases outcome. Induction treatment comprises steroids and cytotoxic agents. In the long term, steroids should be prescribed at the lowest effective dose. They should be maintained for 12 to 24 months and during the second year, at 5 mg/day. Cyclophosphamide is an effective induction treatment. Pulse treatment is recommended for 4 months (6 pulses). Maintenance treatment is based on azathioprine or methotrexate prescribed for 18 to 24 months. In patients presenting with severe renal insufficiency, plasma exchanges improve renal function. Biotherapies now hold a specific place in the therapeutic armamentarium for systemic vasculitides. These therapeutics include polyvalent immunoglobulins, with now quite well defined indications, but also now some more specifically targeted monoclonal antibodies, like anti-TNF-alpha or anti-CD20 for ANCA-associated vasculitides, or anti-IL5 for Churg-Strauss syndrome. However, the exact indications of these latter new agents, as well as their optimal dosage and duration, are not yet precisely defined, mainly for maintenance therapy. Biotherapies are mostly prescribed for patients with disease refractory to conventional therapy. Prospective trials have recently demonstrated that rituximab is as effective as cyclophosphamide to induce remission of granulomatosis with polyangiitis and microscopic polyangiitis. Rituximab is now under evaluation for maintenance therapy. Other biotherapies have been evaluated. If etanercept does not seem effective, to maintain remission, infliximab is effective in severe and refractory ANCA-associated vasculitis. However relapses occur as soon the drug is stopped.

It seems now reasonable to expect dramatic changes in the treatment of ANCA-associated vasculitis with a larger place for biotherapies.