Suspected/Confirmed ANCA-Associated Vasculitis
Clinical features and ANCA positivity suggesting AAV
All Pathways
RheumatologyManagement
ANCA-Associated Vasculitis Management (ACR/VF 2021)
ANCA-Associated Vasculitis Management (ACR/VF 2021): Suspected/Confirmed ANCA-Associated Vasculitis → Confirm Diagnosis → Disease Severity? → Severe GPA...
Pathway Overview
17 steps
Algorithm Steps
17 total
01Start 02Action Confirm Diagnosis
Establish AAV type and severity
- ANCA testing: PR3-ANCA (c-ANCA) vs MPO-ANCA (p-ANCA)
- Tissue biopsy when feasible (kidney, lung, sinus, skin)
- Classify: GPA, MPA, or EGPA
- Assess organ involvement: kidney, lung, ENT, skin, neuro, cardiac
03Decision Disease Severity?
Severe vs non-severe manifestations
- SEVERE: Life/organ-threatening (DAH, RPGN, cardiac, CNS, GI, ocular)
- NON-SEVERE: No immediate threat to life or organ function
- EGPA assessed separately due to different management
04Action Severe GPA/MPA: Remission Induction
Strong recommendation
- Rituximab PREFERRED over cyclophosphamide (Strong)
- RTX: 375 mg/m² weekly x4 OR 1000mg x2 (days 0, 14)
- PLUS glucocorticoids: prednisone 1 mg/kg/day (max 80mg) or IV pulse
- Alternative: Cyclophosphamide (oral or IV) + GC
05Action Glucocorticoid Protocol
Taper to minimize toxicity
- Start: Prednisone 1 mg/kg/day (max 80mg) OR IV pulse 500-1000mg x3 days
- Reduce by ~25% per 2 weeks initially
- Target: ≤10 mg/day by 3-6 months
- Goal: Discontinue or ≤5 mg/day if possible
- May use avacopan (C5a inhibitor) to reduce GC exposure
06Warning ⚠️ Infection Prophylaxis
During remission induction
- PCP prophylaxis: TMP-SMX DS 3x/week (Strong)
- Consider PPI for GI protection
- Vaccinations before rituximab if possible
- Monitor for infections closely
07Decision Remission Achieved?
Absence of active vasculitis signs/symptoms
08Action Remission Maintenance
For GPA/MPA (Strong recommendations)
- Rituximab PREFERRED for GPA/MPA maintenance (Strong)
- RTX: 500mg q6mo OR 1000mg q4-6mo (various protocols)
- Duration: At least 2 years; longer if relapse risk
- Alternative: Azathioprine or MTX (if RTX contraindicated)
- Continue GC taper during maintenance
09Outcome AAV Controlled
Sustained remission on maintenance therapy
10Action EGPA Maintenance
Differs from GPA/MPA
- Mepolizumab continuation for relapsing/steroid-dependent
- Alternative: Azathioprine, MTX, or MMF + low-dose GC
- Monitor for asthma/sinusitis control
- Cardiac follow-up if prior involvement
- Path rejoins step 09Shared downstream outcome
11Action Refractory/Relapsing Disease
Failure of standard induction or maintenance
- Switch rituximab ↔ cyclophosphamide
- Increase rituximab frequency
- Consider avacopan if not already used
- Plasma exchange for severe renal/pulmonary disease
- Clinical trial or specialist vasculitis center
12Warning Vasculitis Specialist Referral
Refractory disease, life-threatening manifestations, complex cases
13Warning ⚠️ Avacopan (Tavneos)
C5aR inhibitor - FDA approved for severe AAV
- 30 mg PO BID with rituximab or cyclophosphamide
- Allows reduced glucocorticoid exposure
- Monitor LFTs (hepatotoxicity risk)
- Alternative for GC-sparing in appropriate patients
- Path rejoins step 07Shared downstream outcome
14Action Non-Severe GPA/MPA: Remission Induction
Conditional recommendation
- Rituximab OR methotrexate + glucocorticoids
- MTX: 15-25 mg/week (if no significant renal impairment)
- Consider organ involvement and patient factors
- GC taper similar to severe disease
- Path rejoins step 05Shared downstream outcome
15Decision EGPA: Severe or Non-Severe?
Eosinophilic GPA has distinct management
16Action Severe EGPA
Life/organ-threatening manifestations
- Rituximab OR cyclophosphamide + glucocorticoids
- High-dose glucocorticoids initially
- Consider mepolizumab after initial control
- Address cardiac involvement urgently
- Path rejoins step 05Shared downstream outcome
17Action Non-Severe EGPA
Conditional recommendation
- Mepolizumab (anti-IL5) PREFERRED (Conditional)
- Mepolizumab 300mg SC q4 weeks
- Plus glucocorticoids (enables GC reduction)
- Alternative: MTX or azathioprine + GC
- Path rejoins step 05Shared downstream outcome
Guideline Source
2021 ACR/Vasculitis Foundation Guideline for the Management of ANCA-Associated Vasculitis
Clinical Safety Information
Clinical Decision Support — Not a Substitute for Clinical Judgment
Individual patient factors may require deviation from these recommendations.
Known Limitations
- Life-threatening manifestations (DAH, RPGN) require emergent specialist input
- Drug-induced AAV not addressed
- Limited guidance for refractory disease
- Avacopan availability varies by region
- Does not cover secondary vasculitis
Applicable Regions
USEUAU
AU: ARA endorses ACR/EULAR recommendations
EU: EULAR 2022 also available
US: ACR/VF 2021 is primary guidance; avacopan FDA-approved
Version 1Next review: 2027-01-01
Next steps
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Related Resources
Frequently Asked Questions
What is the ANCA-Associated Vasculitis Management (ACR/VF 2021)?
The ANCA-Associated Vasculitis Management (ACR/VF 2021) is a management clinical algorithm for Rheumatology. It provides a structured decision tree to guide clinical decision-making, based on 2021 ACR/Vasculitis Foundation Guideline for the Management of ANCA-Associated Vasculitis.
What guideline is the ANCA-Associated Vasculitis Management (ACR/VF 2021) based on?
This algorithm is based on 2021 ACR/Vasculitis Foundation Guideline for the Management of ANCA-Associated Vasculitis (DOI: 10.1002/art.41773).
What are the limitations of the ANCA-Associated Vasculitis Management (ACR/VF 2021)?
Known limitations include: Life-threatening manifestations (DAH, RPGN) require emergent specialist input; Drug-induced AAV not addressed; Limited guidance for refractory disease; Avacopan availability varies by region; Does not cover secondary vasculitis. Individual patient factors may require deviation from these recommendations.
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