title: [Treatment of ANCA-positive systemic vasculitis with intravenous immunoglobins].
abstract: OBJECTIVE
  ANCA positive systemic vasculitis comprises Wegener granulomatosis (WG), microscopic polyangiitis (MPA) and Churg Strauss syndrome (CSS). In WG, anti-Pr3 ANCA are present in 90% of the cases, whereas in MPA and CSS ANCA are present in 40 to 80% and 25 to 60% of the cases, respectively, with anti-MPO specificity. The treatment of WG and MPA associates prednisone and cyclophosphamide, and clinical remission is obtained in 70 to 90% of the cases. However, relapses occur in 10 to 30% of the patients and tolerance of corticosteroids and immunosuppressive therapy is not always good.
  
  
  BACKGROUND
  Intravenous immunoglobulins (IVIg) are well tolerated and indicated in a large number of autoimmune and systemic inflammatory diseases. In vitro IVIg specifically inhibit ANCA activity through V-region dependent interaction of anti-ANCA anti-idiotypic antibodies with ANCA. Several open studies and a few case reports of patients with WG or MPA treated with IVIg were published in the past seven years, with various results.
  
  
  METHODS
  A prospective controlled multicenter study is necessary to evaluate the efficacy of IVIg in the treatment of ANCA positive vasculitides.
authors.affiliation: Service de médecine interne, Hôpital Avicenne, université Paris-Nord, Bobigny, France.
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001167:Arteritis / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D015267:Churg-Strauss Syndrome / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000276:immunology / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use; D012074:Remission Induction / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011241:Prednisone