title: Intravenous Immunoglobulin as an Immunomodulating Agent in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: A French Nationwide Study of Ninety-Two Patients.
abstract: OBJECTIVE
  Intravenous immunoglobulin (IVIG) represents a therapeutic alternative in antineutrophil cytoplasmic antibody-associated vasculitides (AAV), but its efficacy has been evaluated in only 2 small prospective trials. The aim of this study was to evaluate the efficacy and safety of IVIG in patients with AAV.
  
  
  METHODS
  We conducted a nationwide retrospective study of patients who received IVIG as immunomodulatory therapy for AAV.
  
  
  RESULTS
  A total of 92 patients (mean age 51 years) presenting with either granulomatosis with polyangiitis (Wegener's) (68%), eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (22%), or microscopic polyangiitis (10%) received at least 1 course of IVIG. Antineutrophil cytoplasmic antibodies were present in 72% during the flare that required IVIG, as determined by immunofluorescence assay. IVIG was initiated because of relapsing disease in 83% of cases. IVIG was given for a median of 6 months (range 1-156 months) and in combination with corticosteroids in 21% of the patients or with other immunosuppressive agents in 77%. Efficacy of IVIG was assessed in the entire population and in a subset of 34 patients with unmodified background therapy. Remission rates at 6 months were 56% in the entire population and 58% in the unmodified background therapy group. Refractory disease and treatment failure at 6 months were observed in 7% and 18% in the whole population and 3% and 21% in the unmodified background therapy group, respectively. Adverse events (AEs) occurred in 33%, including serious AEs in 12% and AEs leading to discontinuation of IVIG in 7%.
  
  
  CONCLUSIONS
  This large study shows the clinical benefit of IVIG as adjunctive therapy, with an acceptable tolerance profile, and thus supports its use in AAV patients with refractory or relapsing disease.
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Etienne
authors.initials: E
authors.lastname: Crickx
authors.affiliation: Centre Hospitalier Universitaire (CHU) de Bordeaux, Bordeaux, France.
authors.forename: Irène
authors.initials: I
authors.lastname: Machelart
authors.affiliation: Centre Hospitalier Universitaire (CHU) de Bordeaux, Bordeaux, France.
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Lazaro
authors.affiliation: Hôpital Foch, Suresnes, France.
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.affiliation: Groupe Hospitalier Pitié Salpêtrière, AP-HP, and Université Pierre et Marie Curie, Paris, France.
authors.forename: Fleur
authors.initials: F
authors.lastname: Cohen-Aubart
authors.affiliation: CHU de Strasbourg and National Referral Center for Rare Autoimmune and Systemic Diseases, Strasbourg, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Martin
authors.affiliation: CHU Gabriel-Montpied, Clermont-Ferrand, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Mania
authors.affiliation: Centre Hospitalier Claude Huriez, Lille, France.
authors.forename: Pierre-Yves
authors.initials: PY
authors.lastname: Hatron
authors.affiliation: Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France.
authors.forename: Gilles
authors.initials: G
authors.lastname: Hayem
authors.affiliation: Centre Hospitalier de Niort, Niort, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Blanchard-Delaunay
authors.affiliation: CHU de Brest, Brest, France.
authors.forename: Claire
authors.initials: C
authors.lastname: de Moreuil
authors.affiliation: CHU Estaing, Clermont-Ferrand, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.affiliation: Hôpital Erasme, Brussels, Belgium.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Vandergheynst
authors.affiliation: Hôpitaux Privés de Metz, Metz, France.
authors.forename: François
authors.initials: F
authors.lastname: Maurier
authors.affiliation: Hôpital Bichat, AP-HP, Paris, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Crestani
authors.affiliation: Hôpital Avicenne, AP-HP, Bobigny, France.
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.affiliation: CHU de Caen, Caen, France.
authors.forename: Nicolas Martin
authors.initials: NM
authors.lastname: Silva
authors.affiliation: CHU de Caen, Caen, France.
authors.forename: Yann
authors.initials: Y
authors.lastname: Ollivier
authors.affiliation: Centre Hospitalier Intercommunal Eure et Seine, Evreux, France.
authors.forename: Anas
authors.initials: A
authors.lastname: Mehdaoui
authors.affiliation: Hôpital Henri Mondor, AP-HP, Créteil, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Godeau
authors.affiliation: Hôpitaux Universitaires Paris-Sud, AP-HP, Université Paris-Sud, Le Kremlin-Bicêtre, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.affiliation: Hôpital Tenon, AP-HP, Paris, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Cadranel
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Le Jeunne
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, AP-HP, and Université Paris Descartes, Paris, France.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000188:drug therapy; D003345:Corticosterone / Q000008:administration & dosage; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007108:Immune Tolerance; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000009:adverse effects / Q000494:pharmacology*; D056747:Immunomodulation; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D055953:Microscopic Polyangiitis / Q000188:drug therapy; D008875:Middle Aged; D012074:Remission Induction; D012189:Retrospective Studies; D017211:Treatment Failure; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D003345:Corticosterone