Traitement des vascularites systémiques associées aux ANCA

摘要: Key points Treatment for ANCA-associated vasculitides is now well defined, but must be ajusted each patient according to the type of vasculitis, its precise form (e.g., limited versus systemic Wegener's granulomatosis) and severity, patients' characteristics, such as age renal function. The therapeutic decision also take into account risk adverse events inherent treatment. efficacy adequate induction treatment has been demonstrated: more than 80% patients achieve remission. Relapse rates nonetheless remain high, especially in granulomatosis. Patients with microscopic polyangiitis or Churg-Strauss syndrome no poor prognostic factors can treated corticosteroids alone, immunosuppressants added only case failure. granulomatosis one receive a combination immunosuppressants, mainly intravenous pulsed cyclophosphamide. Plasma exchange indicated an adjuvant therapy severe involvement. Once remission achieved, maintenance replace cyclophosphamide by less toxic immunosuppressive drug, azathioprine methotrexate. For these latter patients, optimal duration remains determined, should not shorter 18 months. Conversely, there need prescribe high-dose Prednisone started at 1 mg/kg/d then rapidly tapered so that are receiving 15 mg/d after 3-4 months therapy. Biological therapies appear have place armamentarium vasculitides, least whose disease refractory conventional However, indications anti-TNFα anti-CD20 monoclonal antibodies their regimens (doses durations) yet defined. Anti-IL5, interferon-α anti-IgE might useful syndrome. These biologics prescribed extremely cautiously trial settings, view effects, few severe, recently reported them.