Clinical Outcomes of Treatment of Anti-Neutrophil Cytoplasmic Antibody (ANCA)-associated Vasculitis Based on ANCA Type

Ann Rheum Dis. 2016 Jun;75(6):1166-9. doi: 10.1136/annrheumdis-2015-208073. Epub 2015 Nov 30.

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.

Trial registration number: NCT00104299; post-results.

Keywords: Cyclophosphamide; Granulomatosis with polyangiitis; Systemic vasculitis; Treatment.

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Aged
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / drug therapy*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Azathioprine / therapeutic use
  • Biomarkers / blood
  • Cyclophosphamide / therapeutic use
  • Double-Blind Method
  • Drug Therapy, Combination
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Male
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / drug therapy
  • Microscopic Polyangiitis / immunology
  • Middle Aged
  • Myeloblastin / immunology
  • Peroxidase / immunology
  • Prognosis
  • Remission Induction
  • Rituximab / therapeutic use
  • Treatment Outcome

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Biomarkers
  • Rituximab
  • Cyclophosphamide
  • Peroxidase
  • Myeloblastin
  • Azathioprine

Associated data

  • ClinicalTrials.gov/NCT00104299