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

Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type.

Author information

1
Massachusetts General Hospital, Boston, Massachusetts, USA.
2
Rho, Chapel Hill, North Carolina, USA.
3
University of Pennsylvania School of Medicine, Philadelphia, USA.
4
Hospital for Special Surgery, New York, New York, USA.
5
Johns Hopkins University, Baltimore, Maryland, USA.
6
Cleveland Clinic, Cleveland, Ohio, USA.
7
University of Groningen and University Medical Center Groningen, Groningen, The Netherlands.
8
Duke University Medical Center, Durham, North Carolina, USA.
9
Immune Tolerance Network, San Francisco, California, USA.
10
National Institute of Allergy & Infectious Disease/Division of Allergy, Immunology, & Transplantation (NIAID/DAIT), Bethesda, Maryland, USA.
11
Genentech, South San Francisco, California, USA.
12
Boston University School of Medicine, Boston, Massachusetts, USA.
13
Mayo Clinic, Rochester, Minnesota, USA.

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

PMID:
26621483
PMCID:
PMC4908815
DOI:
10.1136/annrheumdis-2015-208073
[Indexed for MEDLINE]
Free PMC Article

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