ACR Treatment and Management of ANCA-Associated Vasculitis Guideline Summary - Guideline Central

Design and created by Guideline Central in participation with the American College of Rheumatology.

Document Overview

Document Title: Treatment and Management of ANCA-Associated Vasculitis

Authoring Society: American College of Rheumatology

Document Publication Date: Jul 7, 2021

Page Last Reviewed/Updated: May 5, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: onlinelibrary.wiley.com/doi/10.1002/acr.24634

Source Citation: Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis & Rheumatology, Vol. 73, No. 8, August 2021, pp 1366–1383. doi 10.1002/art.41773

Document Scope, Criteria, and Use Cases

Document Objectives: To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody–associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

Scope: Assessment and Screening, Management, Treatment

Diseases/Conditions (MeSH)

D056653 - Rheumatoid Vasculitis

D056647 - Systemic Vasculitis

D056648 - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

D014657 - Vasculitis

D014890 - Granulomatosis with Polyangiitis

D013700 - Giant Cell Arteritis

D056653 - Rheumatoid Vasculitis

D056647 - Systemic Vasculitis

D056648 - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

D014657 - Vasculitis

D014890 - Granulomatosis with Polyangiitis

D013700 - Giant Cell Arteritis

Keywords: ANCA, Antineutrophil Cytoplasmic Antibody, GCA, giant cell arteritis, polyangiitis, vasculitis

Target Patient Population: Patients with vasculitis

Inclusion Criteria: Male, Female, Adolescent, Adult, Child, Infant, Older Adult

Health Care Settings: Ambulatory

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

PICO Questions

In patients with GPA or MPA, what is the impact of obtaining ANCA levels/titers at fixed intervals vs. not obtaining ANCA levels/titers on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of using pulse intravenous vs. high-dose oral glucocorticoids for remission induction on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of using high-dose vs. moderate dose oral glucocorticoids for remission induction on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of using rituximab vs. cyclophosphamide for remission induction on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of using IV CYC vs. po CYC for remission induction on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of initiating treatment with rituximab 1000 mg IV days 1 and 15 vs. rituximab 375 mg/m2 qweek x 4 weeks on disease-related outcomes and treatment-related adverse events?
In patients with active severe GPA/MPA, what is the impact of using avacopan + cyclophosphamide/rituximab vs. cyclophosphamide/rituximab + steroids alone on disease-related outcomes and treatment-related adverse events.
In patients with active non-severe GPA , what is the impact of initiating treatment with azathioprine + glucocorticoids vs. methotrexate + glucocorticoids on disease-related outcomes and treatment-related adverse events?
In patients with active non-severe GPA, what is the impact of initiating treatment with methotrexate + glucocorticoids vs. MMF + glucocorticoids on disease-related outcomes and treatment-related adverse events?
In patients with active non-severe GPA, what is the impact of initiating treatment with glucocorticoids plus either SMZ/TMP vs. methotrexate or azathioprine on disease related outcomes and treatment-related adverse events?
In patients with active non-severe GPA, what is the impact of initiating treatment with methotrexate or azathioprine vs. cyclophosphamide on disease-related outcomes and treatment-related adverse events?
In patients with active non-severe GPA, what is the impact of initiating treatment with methotrexate or azathioprine vs. glucocorticoids on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission, what is the impact of using methotrexate vs. azathioprine for remission maintenance on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission with cyclophosphamide therapy, what is the impact of using rituximab vs. methotrexate or azathioprine for remission maintenance on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission with rituximab therapy, what is the impact of using rituximab vs. methotrexate or azathioprine for remission maintenance on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission with cyclophosphamide or rituximab therapy, what is the impact of using rituximab 1000 mg IV q4 months vs. rituximab 1000 mg IV q6 months vs. rituximab 500 mg IV q6 months for remission maintenance on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission with cyclophosphamide or rituximab therapy, what is the impact of using MMF for remission maintenance vs. methotrexate or azathioprine on disease-related outcomes and treatment-related adverse events?
In patients with severe GPA or MPA who have entered remission with cyclophosphamide or rituximab therapy, what is the impact of using LEF for remission maintenance vs. methotrexate or azathioprine on disease-related outcomes and treatment-related adverse events?

Supplemental Methodology Resources: Evidence Tables Project Plan Project Plan

Number of Source Documents: 55

Includes peer/external review process?: Yes

Includes public comment process?: No

Methodologist involvement?: Yes

Patient involvement?: Yes

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses funding source?: Yes

Discloses conflicts of interest?: Yes

Includes benefits/harms analysis with recommendations?: No