Vasculitis
Also known as: ANCA vasculitis · GCA · giant cell arteritis · PMR · polymyalgia rheumatica
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2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis
American College of Rheumatology (ACR) and Vasculitis Foundation (VF) · 2021 · 2021 guideline
The ACR vasculitis guideline page hosting the 2021 ACR/Vasculitis Foundation guideline series. Alongside ANCA-associated vasculitis, the same 2021 series covers giant cell arteritis and Takayasu arteritis, polyarteritis nodosa, and Kawasaki disease, with plain-language patient versions available through the Vasculitis Foundation.
EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update
European Alliance of Associations for Rheumatology (EULAR) · 2024 · 2022 update
European recommendations on ANCA-associated vasculitis, published in Annals of the Rheumatic Diseases. They cover diagnostic work-up, remission induction and maintenance strategies, glucocorticoid tapering, and management of relapsing or refractory disease across granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis.
- Provides four overarching principles and 17 recommendations. source
- Recommends biopsy and ANCA testing to support diagnosis, and structured glucocorticoid tapering during induction. source
- Addresses newer agents including avacopan for glucocorticoid sparing and mepolizumab in relapsing or refractory eosinophilic granulomatosis with polyangiitis. source
US vs EU key differences
- Induction preference differs: the ACR/VF guideline conditionally prefers rituximab over cyclophosphamide for active severe GPA/MPA, while EULAR treats rituximab and cyclophosphamide as equally acceptable partners to high-dose glucocorticoids and reserves an explicit rituximab preference for relapsing disease. source
- Avacopan is covered only by the European document, which endorses it as a strategy to reduce glucocorticoid exposure in GPA or MPA; the 2021 ACR/VF guideline was written before FDA approval and makes no recommendation on it. source
- Plasma exchange guidance differs in precision: EULAR gives a numeric trigger of serum creatinine above 300 micromol/L, whereas ACR/VF conditionally recommends against routine use and leaves it to case-by-case judgement in active glomerulonephritis or critically ill non-responders. source
- Maintenance duration is specified in Europe but not in the US: EULAR recommends continuing maintenance for at least 24 months, extending toward 48 months in higher-risk patients, while ACR/VF leaves duration to clinical condition and patient preference as an ungraded position. source
- Glucocorticoid tapering targets differ in explicitness: EULAR adopts the PEXIVAS reduced-dose regimen with a target of 5 mg prednisolone equivalent per day by 4-5 months and finds no compelling evidence for intravenous methylprednisolone pulses. source
What changed recently
- EU2016 update → 2022 update2024-01-02
The update added a concrete glucocorticoid tapering target of about 5 mg prednisolone equivalent daily by four to five months, and incorporated avacopan as a steroid-sparing strategy in granulomatosis with polyangiitis and microscopic polyangiitis. Rituximab was recommended for remission maintenance in GPA/MPA, and mepolizumab was added for relapsing or refractory eosinophilic GPA.
source ↗curation confidence 90%
See the full update feed for changes across every system.