title: Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type.
abstract: OBJECTIVE
  To evaluate whether the classification of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) according to ANCA type (anti-proteinase 3 (PR3) or anti-myeloperoxidase (MPO) antibodies) predicts treatment response.
  
  
  METHODS
  Treatment responses were assessed among patients enrolled in the Rituximab in ANCA-associated Vasculitis trial according to both AAV diagnosis (granulomatosis with polyangiitis (GPA)/microscopic polyangiitis (MPA)) and ANCA type (PR3-AAV/MPO-AAV). Complete remission (CR) was defined as disease activity score of 0 and successful completion of the prednisone taper.
  
  
  RESULTS
  PR3-AAV patients treated with rituximab (RTX) achieved CR at 6 months more frequently than did those randomised to cyclophosphamide (CYC)/azathioprine (AZA) (65% vs 48%; p=0.04). The OR for CR at 6 months among PR3-AAV patients treated with RTX as opposed to CYC/AZA was 2.11 (95% CI 1.04 to 4.30) in analyses adjusted for age, sex and new-onset versus relapsing disease at baseline. PR3-AAV patients with relapsing disease achieved CR more often following RTX treatment at 6 months (OR 3.57; 95% CI 1.43 to 8.93), 12 months (OR 4.32; 95% CI 1.53 to 12.15) and 18 months (OR 3.06; 95% CI 1.05 to 8.97). No association between treatment and CR was observed in the MPO-AAV patient subset or in groups divided according to AAV diagnosis.
  
  
  CONCLUSIONS
  Patients with PR3-AAV respond better to RTX than to CYC/AZA. An ANCA type-based classification may guide immunosuppression in AAV.
  
  
  BACKGROUND
  NCT00104299; post-results.
authors.affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Unizony
authors.affiliation: Rho, Chapel Hill, North Carolina, USA.
authors.forename: Miguel
authors.initials: M
authors.lastname: Villarreal
authors.affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Eli M
authors.initials: EM
authors.lastname: Miloslavsky
authors.affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Na
authors.initials: N
authors.lastname: Lu
authors.affiliation: University of Pennsylvania School of Medicine, Philadelphia, USA.
authors.forename: Peter A
authors.initials: PA
authors.lastname: Merkel
authors.affiliation: Hospital for Special Surgery, New York, New York, USA.
authors.forename: Robert
authors.initials: R
authors.lastname: Spiera
authors.affiliation: Johns Hopkins University, Baltimore, Maryland, USA.
authors.forename: Philip
authors.initials: P
authors.lastname: Seo
authors.affiliation: Cleveland Clinic, Cleveland, Ohio, USA.
authors.forename: Carol A
authors.initials: CA
authors.lastname: Langford
authors.affiliation: Cleveland Clinic, Cleveland, Ohio, USA.
authors.forename: Gary S
authors.initials: GS
authors.lastname: Hoffman
authors.affiliation: University of Groningen and University Medical Center Groningen, Groningen, The Netherlands.
authors.forename: Cg M
authors.initials: CM
authors.lastname: Kallenberg
authors.affiliation: Duke University Medical Center, Durham, North Carolina, USA.
authors.forename: E William
authors.initials: EW
authors.lastname: St Clair
authors.affiliation: Rho, Chapel Hill, North Carolina, USA.
authors.forename: David
authors.initials: D
authors.lastname: Ikle
authors.affiliation: Immune Tolerance Network, San Francisco, California, USA.
authors.forename: Nadia K
authors.initials: NK
authors.lastname: Tchao
authors.affiliation: National Institute of Allergy & Infectious Disease/Division of Allergy, Immunology, & Transplantation (NIAID/DAIT), Bethesda, Maryland, USA.
authors.forename: Linna
authors.initials: L
authors.lastname: Ding
authors.affiliation: Genentech, South San Francisco, California, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Brunetta
authors.affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: Hyon K
authors.initials: HK
authors.lastname: Choi
authors.affiliation: Boston University School of Medicine, Boston, Massachusetts, USA.
authors.forename: Paul A
authors.initials: PA
authors.lastname: Monach
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Fernando
authors.initials: F
authors.lastname: Fervenza
authors.affiliation: Massachusetts General Hospital, Boston, Massachusetts, USA.
authors.forename: John H
authors.initials: JH
authors.lastname: Stone
authors.affiliation: Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Ulrich
authors.initials: U
authors.lastname: Specks
mesh_terms: D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D001379:Azathioprine / Q000627:therapeutic use; D015415:Biomarkers / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use; D004311:Double-Blind Method; D004359:Drug Therapy, Combination; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D011379:Prognosis; D012074:Remission Induction; D000069283:Rituximab / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D000069283:Rituximab; D003520:Cyclophosphamide; D009195:Peroxidase; D053146:Myeloblastin; D001379:Azathioprine
keywords: Cyclophosphamide; Granulomatosis with polyangiitis; Systemic vasculitis; Treatment