Title
Treatment and Management of ANCA-Associated Vasculitis
Authoring Organization
Publication Month/Year
July 7, 2021
Last Updated Month/Year
December 4, 2024
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
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).
Target Patient Population
Patients with vasculitis
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?
Inclusion Criteria
Male, Female, Adolescent, Adult, Child, Infant, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Assessment and screening, Treatment, Management
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
Keywords
vasculitis, ANCA, Antineutrophil Cytoplasmic Antibody, polyangiitis, GCA, giant cell arteritis
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