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Research ArticleVasculitis

CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update

Arielle Mendel, Daniel Ennis, Ellen Go, Volodko Bakowsky, Corisande Baldwin, Susanne M. Benseler, David A. Cabral, Simon Carette, Marie Clements-Baker, Alison H. Clifford, Jan Willem Cohen Tervaert, Gerard Cox, Natasha Dehghan, Christine Dipchand, Navjot Dhindsa, Leilani Famorca, Aurore Fifi-Mah, Stephanie Garner, Louis-Philippe Girard, Clode Lessard, Patrick Liang, Damien Noone, Jean-Paul Makhzoum, Nataliya Milman, Christian A. Pineau, Heather N. Reich, Maxime Rhéaume, David B. Robinson, Dax G. Rumsey, Tanveer E. Towheed, Judith Trudeau, Marinka Twilt, Elaine Yacyshyn, Rae S.M. Yeung, Lillian B. Barra, Nader Khalidi and Christian Pagnoux
The Journal of Rheumatology April 2021, 48 (4) 555-566; DOI: https://doi.org/10.3899/jrheum.200721
Arielle Mendel
1A. Mendel, MD, MSc, C.A. Pineau, MD, Division of Rheumatology, Lupus and Vasculitis Clinic, McGill University, Montréal, Québec;
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  • For correspondence: arielle.mendel@mcgill.ca
Daniel Ennis
2D. Ennis, MD, C. Baldwin, MD, N. Dehghan, MD, N. Dhindsa, MD, Division of Rheumatology, University of British Columbia, Vancouver, British Columbia;
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Ellen Go
3E. Go, MD, R.S. Yeung, MD, PhD, Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Ontario;
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Volodko Bakowsky
4V. Bakowsky, MD, Division of Rheumatology, QEII Health Sciences Centre, Dalhousie University, Halifax, Nova Scotia;
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Corisande Baldwin
2D. Ennis, MD, C. Baldwin, MD, N. Dehghan, MD, N. Dhindsa, MD, Division of Rheumatology, University of British Columbia, Vancouver, British Columbia;
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Susanne M. Benseler
5S.M. Benseler, MD, PhD, M. Twilt, MD, PhD, Division of Rheumatology, Department of Pediatrics, Alberta Children’s Hospital, Alberta Children’s Hospital Research Institute, University of Calgary, Calgary, Alberta;
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David A. Cabral
6D.A. Cabral, MBBS, Division of Pediatric Rheumatology, BC Children’s Hospital, University of British Columbia, Vancouver, British Columbia;
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Simon Carette
7S. Carette, MD, MPhil, C. Pagnoux, MD, MSc, MPH, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario;
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Marie Clements-Baker
8M. Clements-Baker, MD, T.E. Towheed, MD, MS, Division of Rheumatology, Queen’s University, Kingston, Ontario;
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Alison H. Clifford
9A.H. Clifford, MD, J.W. Cohen Tervaert, MD, PhD, E. Yacyshyn, MD, Division of Rheumatology, University of Alberta, Edmonton, Alberta;
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Jan Willem Cohen Tervaert
9A.H. Clifford, MD, J.W. Cohen Tervaert, MD, PhD, E. Yacyshyn, MD, Division of Rheumatology, University of Alberta, Edmonton, Alberta;
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Gerard Cox
10G. Cox, MB, Firestone Institute for Respiratory Health, McMaster University, Hamilton, Ontario;
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Natasha Dehghan
2D. Ennis, MD, C. Baldwin, MD, N. Dehghan, MD, N. Dhindsa, MD, Division of Rheumatology, University of British Columbia, Vancouver, British Columbia;
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Christine Dipchand
11C. Dipchand, MD, MSc, Division of Nephrology, QEII Health Sciences Centre, Dalhousie University, Halifax, Nova Scotia;
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Navjot Dhindsa
2D. Ennis, MD, C. Baldwin, MD, N. Dehghan, MD, N. Dhindsa, MD, Division of Rheumatology, University of British Columbia, Vancouver, British Columbia;
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Leilani Famorca
12L. Famorca, MD, S. Garner, MD, MSc, N. Khalidi, MD, Division of Rheumatology, McMaster University, Hamilton, Ontario;
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Aurore Fifi-Mah
13A. Fifi-Mah, MD, Division of Rheumatology, University of Calgary, Calgary, Alberta;
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Stephanie Garner
12L. Famorca, MD, S. Garner, MD, MSc, N. Khalidi, MD, Division of Rheumatology, McMaster University, Hamilton, Ontario;
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Louis-Philippe Girard
14L.P. Girard, MD, MSc, Division of Nephrology, University of Calgary, Calgary, Alberta;
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Clode Lessard
15C. Lessard, MD, Centre de Recherche Musculo-Squelettique, Trois-Rivières, Québec;
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Patrick Liang
16P. Liang, MD, Division of Rheumatology, Centre Hospitalier Universitaire de Sherbrooke, Université de Sherbrooke, Sherbrooke, Québec;
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Damien Noone
17D. Noone, MB, BCh, BAO, MSc, Division of Nephrology, Hospital for Sick Children, University of Toronto, Toronto, Ontario;
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Jean-Paul Makhzoum
18J.P. Makhzoum, MD, M. Rhéaume, MD, Division of Internal Medicine, Hôpital du Sacré-Coeur de Montréal, Université de Montréal, Montréal, Québec;
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Nataliya Milman
19N. Milman, MD, MSc, Division of Rheumatology, The Ottawa Hospital, University of Ottawa and Ottawa Hospital Research Institute, Ottawa, Ontario;
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Christian A. Pineau
1A. Mendel, MD, MSc, C.A. Pineau, MD, Division of Rheumatology, Lupus and Vasculitis Clinic, McGill University, Montréal, Québec;
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Heather N. Reich
20H.N. Reich, MD, PhD, Division of Nephrology, University Health Network, University of Toronto, Toronto, Ontario;
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Maxime Rhéaume
18J.P. Makhzoum, MD, M. Rhéaume, MD, Division of Internal Medicine, Hôpital du Sacré-Coeur de Montréal, Université de Montréal, Montréal, Québec;
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David B. Robinson
21D.B. Robinson, MD, MSc, Section of Rheumatology, University of Manitoba, Winnipeg, Manitoba;
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Dax G. Rumsey
22D.G. Rumsey, MD, MSc, Division of Pediatric Rheumatology, University of Alberta, Edmonton, Alberta;
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Tanveer E. Towheed
8M. Clements-Baker, MD, T.E. Towheed, MD, MS, Division of Rheumatology, Queen’s University, Kingston, Ontario;
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Judith Trudeau
23J. Trudeau, MD, Division of Rheumatology, CISSS Chaudière-Appalaches, Université Laval, Québec City, Québec;
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Marinka Twilt
5S.M. Benseler, MD, PhD, M. Twilt, MD, PhD, Division of Rheumatology, Department of Pediatrics, Alberta Children’s Hospital, Alberta Children’s Hospital Research Institute, University of Calgary, Calgary, Alberta;
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Elaine Yacyshyn
9A.H. Clifford, MD, J.W. Cohen Tervaert, MD, PhD, E. Yacyshyn, MD, Division of Rheumatology, University of Alberta, Edmonton, Alberta;
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Rae S.M. Yeung
3E. Go, MD, R.S. Yeung, MD, PhD, Division of Rheumatology, Hospital for Sick Children, University of Toronto, Toronto, Ontario;
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Lillian B. Barra
24L.B. Barra, MD, Division of Rheumatology, Department of Medicine, Western University, London, Ontario, Canada.
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Nader Khalidi
12L. Famorca, MD, S. Garner, MD, MSc, N. Khalidi, MD, Division of Rheumatology, McMaster University, Hamilton, Ontario;
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Christian Pagnoux
7S. Carette, MD, MPhil, C. Pagnoux, MD, MSc, MPH, Division of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario;
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Abstract

Objective In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. The current update aims to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evidence.

Methods A needs assessment survey of CanVasc members informed questions for an updated systematic literature review (publications spanning May 2014 to September 2019) using Medline, Embase, and Cochrane. New and revised recommendations were developed and categorized according to the level of evidence and strength of each recommendation. The CanVasc working group used a 2-step modified Delphi procedure to reach > 80% consensus on the inclusion, wording, and grading of each new and revised recommendation.

Results Eleven new and 16 revised recommendations were created and 12 original (2015) recommendations were retained. New and revised recommendations are discussed in detail within this document. Five original recommendations were removed, of which 4 were incorporated into the explanatory text. The supplementary material for practical use was revised to reflect the updated recommendations.

Conclusion The 2020 updated recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV in Canada with new management guidance, based on current evidence and consensus from Canadian experts.

Key Indexing Terms:
  • antineutrophil cytoplasm antibody-associated vasculitis
  • eosinophilic granulomatosis with polyangiitis
  • glomerulonephritis
  • granulomatosis with polyangiitis
  • microscopic polyangiitis

The antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are systemic necrotizing vasculitides, classified into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA)1. Advances in the management of AAV have improved patient outcomes, but the burden of organ damage, treatment toxicity, and relapse risk remains high.

The Canadian Vasculitis Research Network (CanVasc) aimed to update the original (2015) AAV management recommendations2 based on the expanding literature of the last 5 years. Full-length recommendations are contained within Supplementary Data 1 (available with the online version of this article). Only new or revised recommendations from the original publication2 are discussed. Supplementary Data 2 contains treatment protocols and other practical guidance.

MATERIALS AND METHODS

The full-length version of the article (Supplementary Data 1, available with the online version of this article) contains detailed methodology. Briefly, an electronic survey distributed to all 34 CanVasc Core members and main associates established 15 primary questions and 4 secondary topics to inform a systematic literature review (Table 1). The literature search (publications spanning May 2014 to September 2019, updated January and March 2020) included guidelines, randomized controlled trials (RCT), systematic reviews and/or metaanalyses, observational studies, and case series of ≥ 15 adult or pediatric patients (literature search and writing committee: AM, lead; DE for EGPA publications; EG for pediatric publications; and CP). The evidence and strength for revised and new recommendations were graded according to European League Against Rheumatism criteria (Table 2)3. Through a modified Delphi consensus procedure, a 36-member working group voted on the inclusion, grading, and wording of each recommendation. Disagreements were resolved in 2 rounds of teleconferences (January and March 2020). Each final recommendation achieved ≥ 80% approval. Reviewers from the Canadian Rheumatology Association Guidelines Committee, Canadian Thoracic Society, and Canadian Glomerulonephritis Registry provided feedback, as did 3 patients. Ethics approval was not required in accordance with the Tri-Council Policy Statement. Supplementary Data 3 contains author disclosures.

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Table 1

Questions informing the literature review for updated recommendations.

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Table 2

Level of evidence and grading of recommendationsa.

RESULTS

I. Updated Diagnostic Recommendations

1. A high-quality, antigen-specific immunoassay for proteinase 3–ANCA and myeloperoxidase-ANCA is the preferred method of ANCA detection for patients in whom there is clinical suspicion of ANCA-associated vasculitis (Category 2A, Strength B). Performance characteristics of antigen-specific immunoassays (first, second, and third generation ELISA, chemiluminescent assay, fluorescence enzyme, and multiplex bead immunoassays, detecting IgG antibodies to proteinase-3 [PR3] and myeloperoxidase [MPO]) were compared to indirect immunofluorescence (IIF) detection of cytoplasmic staining ANCA and perinuclear staining ANCA, using a large cohort of untreated patients with GPA or MPA and controls4. Antigen-specific immunoassays had superior performance to the 2 IIF techniques (area under the curve [AUC] 0.92–0.95 vs 0.84–0.92)4. High-quality, antigen-specific immunoassays are now recommended for PR3- and MPO-ANCA detection in patients with suspected GPA or MPA5. In selected patients with negative antigen-specific immunoassay testing but a high clinical suspicion of AAV, a second assay or IIF can improve sensitivity5.

II. Updated Therapeutic Recommendations

A. Updated recommendations for induction treatment of GPA and MPA

5a. An initial dose of 1 mg/kg/day prednisone equivalent (not exceeding 80 mg/day) is recommended for remission induction in adult patients with severe GPA or MPA. (Category 2A, Strength C). Whereas the optimal glucocorticoid (GC) starting dose in severe AAV is unknown, prednisone 1 mg/kg/day (not exceeding 80 mg/day) has been a common practice6. Patients with nonsevere/limited GPA can start at lower prednisone doses (e.g., 0.5 mg/kg/day)7.

5b. Pulse intravenous methylprednisolone (0.5–1 g/day for 1–3 days) can be considered in severe, organ- or life-threatening GPA or MPA, but lacks proven efficacy and carries a potential risk of adverse effects (Category 3, Strength D).

Intravenous (IV) methylprednisolone (MP) pulses (500–1000 mg/day for 1–3 days) are often administered at the onset of induction therapy in patients with life-threatening AAV2. However, observational data suggest a lack of benefit and potential harm compared to high-dose GC without pulses.

Two retrospective studies of patients with severe AAV (creatinine [Cr] > 500 μmol/L or dialysis] found no difference in 1-year overall survival or renal recovery8,9 and time to renal recovery or relapses8 among IV MP vs non-MP recipients. IV MP was associated with a nearly 3-fold higher risk of serious infection at 3 months, and a 6-fold higher risk of new-onset diabetes mellitus8. Other studies also found IV MP to be independently associated with severe infections10,11 and treatment-related damage12.

IV MP pulses will remain a common practice in severe AAV, but caution should be exercised given the observed excess risk of adverse events and the lack of demonstrated benefit.

6a. A GC tapering protocol should be initiated within 2 weeks of induction therapy in patients with severe GPA or MPA (Category 2A, Strength C).

6b. A reduced-dose GC tapering protocol can be considered in adult patients with severe GPA or MPA who are receiving cyclophosphamide or rituximab induction therapy, to reduce cumulative GC exposure and infection risk (Category 1B, Strength A).

In most patients, GC tapering can commence within 1–2 weeks of commencing induction therapy with rituximab (RTX) or cyclophosphamide (CYC; Supplementary Table 5 and Supplementary Data 2.3, available with the online version of this article)6,13. The PEXIVAS trial13 found that a reduced-dose tapering protocol, which commenced tapering after 1 week and aimed for prednisone 7.5–12.5 mg/day by 3 months, was noninferior to a “standard” regimen reaching 15–25 mg/day by 3 months for the composite endpoint of death or endstage renal disease (ESRD), and resulted in fewer serious infections13. For certain PEXIVAS subgroups, particularly subjects who received RTX induction (15%), the safety and effectiveness of a reduced-dose GC regimen requires further study. GC tapering requires repeated clinical assessments, with modification of protocols according to the clinical status of the patient.

7. In patients with severe, newly diagnosed GPA or MPA, we recommend GC plus either CYC or RTX for first-line remission induction therapy. RTX is preferred for remission induction in patients with severe GPA or MPA in whom CYC is contraindicated, including those with a risk of infertility (Category 1B, Strength A).

A previous metaanalysis of RAVE6 and RITUXVAS14 confirmed no difference between RTX and CYC for remission at 6 months, relapse at 12 months, death, or serious adverse events (SAE) including infections15. Post-hoc analyses of the RAVE trial found that subgroups with relapsing disease16 and PR3-ANCA17 achieved higher remission rates with RTX compared to CYC plus azathioprine (AZA). In relapsing disease following initial CYC induction, RTX is the preferred induction agent (see Recommendation 13 in Table 3). Among patients with severe GPA and MPA who do not present an infertility risk or other contraindications to CYC, both CYC and RTX can be considered as first-line induction therapies. When CYC fails to induce remission, RTX should be used (and vice versa). Observational data suggest that RTX-treated patients with very severe renal disease have similar outcomes to those treated with CYC18. Supplementary Data 2 (available with the online version of this article) contains CYC administration, fertility, and monitoring guidance.

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Table 3

Updated recommendations: executive summary.

9. Urgent plasma exchange is not recommended as part of initial induction therapy for most adult patients with severe GPA or MPA (Category 1B, Strength A).

PEXIVAS compared plasma exchange to no plasma exchange along with standard treatment in 704 adults with severe AAV13. At a median of 2.9 years’ follow-up, there was no difference in ESRD or death between groups (HR 0.86, 95% CI 0.65–1.13)13. A metaanalysis assessing plasma exchange for the outcome of dialysis at 1 year (not including PEXIVAS data) found that plasma exchange reduced dialysis risk at 12 months (risk ratio [RR] 0.45, 95% CI 0.29–0.72)15. Metaanalyses including data from PEXIVAS found no difference in sustained remission, total adverse events, or death at any time point, but found increased serious infections with plasma exchange (RR 1.26, 95% CI 1.03–1.54)15.

PEXIVAS included patients with pulmonary hemorrhage (27%) and Cr ≥ 500 μmol/L or dialysis (29%), however some may consider that the effect estimates for these disease subsets remain inconclusive. The possibility that plasma exchange could delay the short-term need for dialysis19 requires further investigation. Although routine use of plasma exchange for induction is not recommended, it may still be considered in these subgroups, in consultation with vasculitis experts. Finally, plasma exchange is required in patients with antiglomerular basement membrane (anti-GBM) antibodies20, and in the absence of timely anti-GBM testing (serology or renal biopsy) initial empiric plasma exchange may be appropriate.

11. In patients with GPA or MPA without life-threatening or extensive disease manifestations, remission induction with mycophenolate mofetil in combination with GC can be considered (Category 1B, Strength A).

Two previous open-label, multicenter RCT compared mycophenolate mofetil (MMF) to IV21 or oral22 CYC for induction, excluding patients with very severe disease. The MYCYC trial21 found noninferiority for remission at 6 months (67% MMF and 61% CYC) and time to remission, but relapse-free survival was lower in the MMF group21. In the second RCT including only relapsing patients (89% PR3–, ANCA+), 6-month remission rates and 2-year disease-free survival were not statistically different between CYC and MMF groups (81% vs 66% and 61% vs 43%, respectively), and more MMF recipients were taking < 10 mg of prednisone at 6 months (62% vs 36%)22. Patients in the highest tertile of disease severity were less likely to respond to MMF22.

A metaanalysis including these 2 studies plus 2 prior RCT from China (primarily MPO-ANCA+ patients with more severe renal disease)23,24, found no difference between MMF or CYC for remission, relapse, SAE, or infection25. In the absence of severe disease (e.g., patients with rash, mild neuropathy, or mild renal involvement), MMF can be used for remission induction in GPA or MPA.

B. Updated recommendations for remission maintenance in GPA and MPA

14. In patients with GPA or MPA who received CYC or RTX induction therapy, RTX (infusions every 4–6 months) is recommended as first-line maintenance therapy (Category 1B, Strength A).

The MAINRITSAN26 trial demonstrated long-term superiority of RTX over AZA for preventing major relapses after CYC induction27. RITAZAREM was an open-label RCT comparing maintenance RTX (1 g every 4 months) to AZA (2 mg/kg/day) among 170 patients with relapsing disease (72% PR3+) after RTX induction28. At month 24, RTX showed superior relapse-free survival compared to AZA (HR 0.36, 95% CI 0.23–0.57), fewer SAE (22% vs 36%), and no difference in infection rates28. No RCT have compared RTX to other maintenance therapies among nonrelapsing patients treated with RTX induction. RTX is not yet funded for GPA and MPA maintenance in every province.

Originator and biosimilar RTX have comparable efficacy and safety in rheumatoid arthritis29. RTX biosimilars could thus be used for induction and maintenance of GPA and MPA if they receive approval.

15a. “Tailored” RTX maintenance, with retreatment based on ANCA titer rise, switch from negative to positive ANCA, or repopulation of CD19+ B cell subsets30, can be an alternative maintenance strategy in adults with GPA or MPA who received CYC or RTX induction therapy (Category 1B, Strength B).

MAINRITSAN2 was an open-label RCT comparing “fixed” maintenance (500 mg at Day 0 and 14, then every 6 months for 18 months) to “tailored” maintenance (500 mg at Day 0 with 500 mg reinfusion if ANCA titers became positive, ELISA value rose 2-fold, IIF titer increased ≥ 2 dilutions, or CD19 B cells rose above 0/mm3; repeating ANCA and CD19 B cells at 3-month intervals)30. Patients in the tailored regimen received fewer infusions with no difference in total or major relapses (17% vs 10% and 7% vs 4%, respectively) or relapse-free survival (84% vs 86%) at Month 2830.

A tailored regimen may reduce RTX cost, although the cost and availability of repeated CD19 B cell and ANCA measurements need to be considered. Tailored RTX maintenance may be useful for patients for whom systematic RTX infusions are not funded or according to patient preference, provided serial ANCA and CD19 B cell monitoring can be performed.

15b. Outside of “tailored” RTX maintenance, there is insufficient evidence to recommend escalating immunosuppressive therapy based on ANCA titer or CD 19 B cell rise (Category 4, Strength D).

Rising ANCA titer31, return to ANCA positivity32,33, or persistently positive ANCA27,34 are associated with increased risk of relapse, whereas ANCA-negative status is associated with decreased relapse risk33,35. While not all relapsing patients have detectable B cells32,36, relapse-free survival was lower among patients who repopulated B cells within 12 months32. MAINRITSAN2, with a small number of total relapses (n = 22), was underpowered to analyze the predictive value of ANCA (rise, return) or B cell repopulation on relapses.

Whereas ANCA trajectory during remission maintenance likely adds predictive value for determining relapse risk, outside of the “tailored” RTX maintenance regimen, there is insufficient evidence that an ANCA rise alone should dictate therapy escalation. Nevertheless, ANCA testing can be useful in stratifying patients’ future flare risk, to inform the frequency of clinical and laboratory follow-up.

16a. In patients with GPA or MPA who received CYC or RTX induction therapy, AZA or methotrexate can be used for maintenance therapy when RTX maintenance cannot be used (Category 1B, Strength B for maintenance after CYC induction; Category 3, Strength C for maintenance after RTX induction).

16b. MMF or leflunomide (LEF) can be considered as alternative maintenance therapies in patients with contraindications, poor tolerance, or lack of response to other agents (Category 1B, Strength B for LEF; Category 3, Strength C for MMF).

Ten-year follow-up from the WEGENT trial found no difference in relapse rates between AZA and methotrexate (MTX) maintenance37. In a network metaanalysis of RCT comparing MTX, AZA, LEF, and MMF for maintenance therapy, estimates for a superior agent were statistically inconclusive, but MMF was overall inferior38. Observational data suggest that patients in stable remission who received MMF induction can continue MMF for maintenance39.

Full-dose trimethoprim-sulfamethoxazole (TMP-SMX; 800/160 mg once or twice daily) can be an adjunctive maintenance agent in selected GPA cases40.

17. In patients with GPA or MPA, maintenance with RTX (or conventional immunosuppressants) should be continued for a minimum of 2 years; extended maintenance therapy can be considered, especially in high-risk clinical subgroups (Category 1B, Strength B).

17.1. Maintenance duration of nonbiologic immunosuppressants following GC-CYC induction. Two RCT34,41 compared “standard” to “extended” maintenance therapy among patients who received CYC induction. In a Dutch study41, relapse-free survival was 52% among patients randomized to taper AZA after 1 year and 72% among those who continued for 4 years, although the difference was not statistically significant41. In the REMAIN trial34, patients randomized to withdraw AZA and prednisone after a mean of 19 months had higher relapse rates (OR 5.96, 95% CI 2.58–13.77) and ESRD (7.8% vs 0%, P = 0.012) compared to those who continued maintenance for an additional 24 months34. A metaanalysis of both studies found a reduced relapse risk with the extended maintenance (RR 0.41, 95% CI 0.26–0.64) with no difference in adverse events15.

In patients with a history of prior relapse, significant preexisting organ damage, or PR3-ANCA and/or persistent ANCA positivity, maintenance durations of at least 4 years should be considered. Beyond 4 years, clinicians should base further maintenance extension on history of relapses, preexisting damage, and patient preference.

17.2. Maintenance duration of RTX. MAINRITSAN342 enrolled 97 patients in remission at month 28 from MAINRITSAN2 (last possible maintenance RTX infusion at Month 18), who were randomized to receive RTX 500 mg every 6 months until 46 months, or placebo. At 56 months, relapse-free survival was 96% in the extended therapy vs 74% in the placebo group (HR 7.5, 95% CI 1.7–33.7) with no difference in SAE42. Hence, while the majority of patients may not require further RTX after 18 months, extended maintenance therapy reduces relapse risk further.

RTX maintenance should continue for 2 years minimum (last infusion at 18 months), but high-risk clinical characteristics (see 17.1 above) support continuing RTX maintenance for an additional 18 months (Supplementary Data 2.3, available with the online version of this article).

C. Updated recommendations for the treatment of EGPA

20a. An initial dose of 1 mg/kg/day prednisone equivalent (not exceeding 80 mg/day) is recommended for remission induction in patients with severe EGPA (Category 2A, Strength C).

20b. Pulse IV MP can be considered in severe, organ- or life-threatening EGPA, but lacks proven efficacy and carries a potential risk of adverse effects (Category 3, Strength D).

In a trial of EGPA patients with poor prognostic factors [i.e., Five Factor Score (FFS) ≥ 1], IV MP pulses were given to 72% of patients43. There are no studies comparing the efficacy of pulse vs no pulse MP for induction of severe EGPA. Until such data are available, recommendations are extrapolated from GPA and MPA (Recommendation 5).

21. A GC tapering protocol should be initiated within 2–4 weeks of induction therapy in EGPA (Category 4, Strength D).

The EGPA Consensus Task Force44 recommends tapering prednisone after 2–3 weeks, to approximately 20 mg/day by 3 months. Unlike in GPA and MPA, a reduced-dose GC taper has not been evaluated in EGPA.

22. We recommend remission induction therapy with a combination of GC and CYC in patients with severe, newly diagnosed EGPA (Category 2A, Strength B).

In a prospective trial of patients with EGPA and FFS ≥ 1, IV CYC pulses led to complete remission in 89% of patients43. Extrapolating from GPA and MPA data34,37,38, CYC induction should be followed by either AZA or MTX maintenance (with LEF or MMF as alternatives) for a minimum of 2 years.

23. Patients with nonsevere EGPA without major organ involvement or poor prognostic factors may be treated with GC alone for initial induction therapy (Category 1B, Strength A).

CHUSPAN2 compared AZA plus GC to GC alone for induction in nonsevere (FFS = 0), newly diagnosed EGPA (n = 51), MPA (n = 25), or polyarteritis nodosa (n = 19)45. Within the EGPA subset at 2 and 5 years, relapse-free survival did not differ between groups46. Although there is no evidence that adding immunosuppressants to initial induction is superior to GC alone, conventional immunosuppressants are often justified if vasculitic disease manifestations, such as mononeuritis multiplex, progress47. Until further data are available, any of the conventional immunosuppressants (AZA, MTX, LEF, MMF, or even CYC in some cases) should be promptly added in patients with progressive vasculitic manifestations of EGPA for whom the FFS remains 0.

24. Mepolizumab 300 mg subcutaneous monthly can be considered in nonsevere, GC-dependent refractory or relapsing EGPA (Category 1B, Strength A).

MIRRA48 compared mepolizumab (MPB; 300 mg subcutaneous every 4 weeks) to placebo in 136 patients with refractory, relapsing, or GC-dependent EGPA (new diagnosis and severe disease excluded). The primary endpoint of remission (Birmingham Vasculitis Activity Score = 0) at Weeks 36 and 48 occurred more often in the MPB group (OR 16.74, 95% CI 3.6–77.6)48. Relapse rates were reduced but remained high overall (56% vs 82% with placebo), with no difference in SAE48. However, MIRRA was unable to determine the efficacy of MPB for acute vasculitic manifestations or myocarditis48. As of yet, no anti–interleukin 5 studies have been completed in patients without relapsing or refractory, GC-dependent EGPA.

25. Consideration of other (off-label) therapies for EGPA should be made in collaboration with centers of expertise (Category 4, Strength D).

Case series have suggested benefit of RTX for patients with relapsing or refractory EGPA49,50. Response or median time to remission may be better in ANCA-positive patients50. A retrospective study comparing 14 RTX recipients to 14 CYC recipients found similar remission (36% vs 29%) and relapse-free survival rates between groups51. While 2 RCT will evaluate RTX for EGPA induction (ClinicalTrials.gov: NCT02807103) and maintenance (ClinicalTrials.gov: NCT03164473), currently RTX should be reserved for patients who have failed conventional therapies and should be discussed with a center of expertise.

D. Updated recommendations for special treatment groups

28. For patients with subglottic and/or bronchial stenosis, multidisciplinary management should be sought to optimize local interventions, and consideration should be given to systemic immunosuppressive therapy (Category 3, Strength C).

Subglottic stenosis occurs in 10–23% of GPA patients52,53 while bronchial stenosis is less common52. Cohort studies have demonstrated efficacy of dilatation procedures, often with intralesional corticosteroid injection, but recurrent stenosis is common, requiring repeated procedures53,54. Treatment with prednisone > 30 mg at the time of dilation was associated with a lower risk of restenosis (HR 0.53, 95% CI 0.31–0.89)55. Based on these limited data, patients with subglottic stenosis and bronchial stenosis may benefit from periprocedural escalation of GC (to > 30 mg/day for 3–5 days), and/or a trial of immunosuppressive therapy.

32. In children with newly diagnosed, severe GPA or MPA, we recommend GC plus either CYC or RTX for remission induction (Category 3, Strength C).

Although data on RTX remains limited in pediatric AAV, RTX may be preferred in children for whom CYC presents an excess risk of toxicity. A phase IIa, multicenter, single-arm trial of RTX induction (375 mg/m2 weekly for 4 doses) was conducted in 25 pediatric patients with new or relapsing GPA and MPA, excluding those with alveolar hemorrhage or hemodialysis56. Remission (Pediatric Vasculitis Activity Score = 0 and prednisone ≤ 0.2 mg/kg/day) was 56% at 6 months and 100% at 18 months with no concerning adverse events56. This outcome is comparable to a cohort of 105 pediatric patients with AAV who primarily received CYC57. In patients with relapsing disease after CYC, RTX is the preferred agent (Table 3, Recommendation 33).

E. Updated recommendations for monitoring and prevention in AAV

36a. All patients previously treated with CYC should have a urinalysis every 3–6 months as a lifelong means of screening for CYC-induced bladder malignancy. If micro- or macroscopic hematuria is present, in the absence of an alternate explanation, the patient should be referred for consideration of a cystoscopy (Category 3, Strength D).

36b. Patients should be counseled on the increased risk of nonmelanoma skin cancer after exposure to CYC and/or other conventional immunosuppressants (Category 3, Strength D).

Patients with AAV and exposure to CYC have an increased risk of nonmelanoma skin cancer58,59,60 that may be accelerated by subsequent long-term AZA59. Studies have demonstrated an increased risk of other malignancies with cumulative CYC exposure > 25–36 g58,59,60, thus cumulative CYC > 25 g should be avoided if possible. Patients exposed to CYC should undergo long-term urinalysis monitoring, and persistent unexplained hematuria evaluated with cystoscopy.

37a. TMP-SMX prophylaxis should be prescribed to prevent infection during induction therapy with CYC or RTX (Category 3, Strength C).

37b. Prophylaxis should continue for at least 3 months following CYC cessation and 6 months following last RTX dose (Category 4, Strength D).

Pneumocystis jirovecii pneumonia prophylaxis with TMP-SMX is recommended in patients receiving CYC or RTX. In the presence of TMP-SMX intolerance or allergy, dapsone or atovaquone are alternatives (Supplementary Data 2.3, available with the online version of this article). Further, an RCT of therapeutic TMP-SMX in GPA40 and a previous observational study of primarily prophylactic dose TMP-SMX in RTX recipients61 found that TMP-SMX reduced overall infection risk in AAV. Although there is little evidence to inform prophylaxis duration, a recommended strategy is continued prophylaxis for 3 months following CYC cessation and 6 months following the last RTX infusion6.

38. Pneumococcal vaccination (Category 3, Strength D) and annual influenza vaccination (Category 1B, Strength B) are recommended in all patients with AAV receiving immunosuppression. Recombinant varicella zoster virus subunit (nonlive) vaccine can be offered to all adult patients at risk (Category 4, Strength D). Patients with AAV undergoing immunosuppression should receive the 13-valent pneumococcal conjugate vaccine followed by the 23-valent pneumococcal polysaccharide vaccine at least 8 weeks later (Supplementary Data 2.4, available with the online version of this article)62,63.

Patients with AAV should also receive annual influenza vaccination and any available nonlive vaccinations to other seasonal and/or pandemic viruses. In an RCT, trivalent influenza immunogenicity among patients with AAV in remission was acceptable (although attenuated compared to controls)64, and did not increase relapse risk64. The high-dose influenza vaccine, which has greater immunogenicity in immunocompromised patients65, requires study in AAV.

Varicella zoster virus (VZV) infections are common in AAV66. The recombinant zoster vaccine should be administered instead of the live, attenuated VZV vaccine in patients taking immunosuppression.

In patients receiving maintenance RTX, vaccines are ideally administered 5 months after the last infusion and 1 month before the upcoming infusion67. If such timing is not feasible, vaccination is still preferred, despite the lower likelihood of immunogenicity.

39. Immunoglobulin levels should be checked in patients receiving RTX who experience serious or recurrent infections (Category 4, Strength D).

Hypogammaglobulinemia secondary to RTX can be associated with severe infections66,68. Risk factors for persistent or severe hypogammaglobulinemia include baseline low IgG68, older age68, and prior CYC69. Ig replacement (e.g., 0.4 g/kg IV Ig/month) is indicated if hypogammaglobulinemia is associated with “serious, persistent, unusual, or recurrent infections”68,69,70.

Patients with hypogammaglobulinemia without recurrent infections can receive repeat RTX infusions with close monitoring. In the event of recurrent or severe infections, further RTX treatment should be individualized based on disease activity and relapse risk, with Ig replacement if needed.

In conclusion, these recommendations, which incorporate the expert consensus of more than 30 physicians with a wide breadth of experience from different Canadian healthcare contexts, provide a guide for applying new evidence to the care of Canadians with AAV, and must be used in conjunction with best clinical judgment in a given patient.

Table 4 lists current high-importance questions for AAV management in Canada. We continue to recommend collaborative multidisciplinary care, including referral to vasculitis centers, especially for patients with AAV in whom diagnostic or therapeutic uncertainty remains.

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Table 4

Clinical research questions of high importance.

ACKNOWLEDGMENT

CanVasc wishes to acknowledge the work of Brandon Lee, Renee Aklass, and Michael Wiensczyk, and members of the Canadian Glomerulonephritis Registry and Canadian Rheumatology Association Guidelines Committee for their critical review of the updated recommendations.

Footnotes

  • VB reports advisory board attendance from AbbVie, Eli Lilly, and Janssen, outside the submitted work. AHC reports other fees from AbbVie and personal fees from Roche, outside the submitted work. JWCT reports Chair of the Independent Data Monitoring Committee for InflaRx, outside the submitted work. ND reports personal fees from Novartis, Sandoz, and Amgen, outside the submitted work. SG reports grants from the Vasculitis Foundation and Roche, during the conduct of the submitted work. PL reports grants from Roche, Amgen, Janssen, AbbVie, BMS, and Novartis, outside the submitted work. JT reports personal fees from Roche, personal fees from Medexus, and other fees from Medpace, outside the submitted work. LB reports honoraria from Roche, Bristol Myers Squibb, Boehringer Ingelheim, and Pfizer, outside the submitted work. NK reports personal fees and nonfinancial support from Roche, and nonfinancial support from Bristol Myers Squibb, outside the submitted work. CP reports grants and personal fees from Roche; grants and personal fees from GSK; and personal fees from ChemoCentryx, Sanofi, and InflaRx GmbH, outside the submitted work. The remaining authors have nothing to disclose.

  • Accepted for publication September 5, 2020.
  • Copyright © 2021 by the Journal of Rheumatology

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CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update
Arielle Mendel, Daniel Ennis, Ellen Go, Volodko Bakowsky, Corisande Baldwin, Susanne M. Benseler, David A. Cabral, Simon Carette, Marie Clements-Baker, Alison H. Clifford, Jan Willem Cohen Tervaert, Gerard Cox, Natasha Dehghan, Christine Dipchand, Navjot Dhindsa, Leilani Famorca, Aurore Fifi-Mah, Stephanie Garner, Louis-Philippe Girard, Clode Lessard, Patrick Liang, Damien Noone, Jean-Paul Makhzoum, Nataliya Milman, Christian A. Pineau, Heather N. Reich, Maxime Rhéaume, David B. Robinson, Dax G. Rumsey, Tanveer E. Towheed, Judith Trudeau, Marinka Twilt, Elaine Yacyshyn, Rae S.M. Yeung, Lillian B. Barra, Nader Khalidi, Christian Pagnoux
The Journal of Rheumatology Apr 2021, 48 (4) 555-566; DOI: 10.3899/jrheum.200721

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CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update
Arielle Mendel, Daniel Ennis, Ellen Go, Volodko Bakowsky, Corisande Baldwin, Susanne M. Benseler, David A. Cabral, Simon Carette, Marie Clements-Baker, Alison H. Clifford, Jan Willem Cohen Tervaert, Gerard Cox, Natasha Dehghan, Christine Dipchand, Navjot Dhindsa, Leilani Famorca, Aurore Fifi-Mah, Stephanie Garner, Louis-Philippe Girard, Clode Lessard, Patrick Liang, Damien Noone, Jean-Paul Makhzoum, Nataliya Milman, Christian A. Pineau, Heather N. Reich, Maxime Rhéaume, David B. Robinson, Dax G. Rumsey, Tanveer E. Towheed, Judith Trudeau, Marinka Twilt, Elaine Yacyshyn, Rae S.M. Yeung, Lillian B. Barra, Nader Khalidi, Christian Pagnoux
The Journal of Rheumatology Apr 2021, 48 (4) 555-566; DOI: 10.3899/jrheum.200721
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Keywords

antineutrophil cytoplasm antibody-associated vasculitis
EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS
GLOMERULONEPHRITIS
GRANULOMATOSIS WITH POLYANGIITIS
MICROSCOPIC POLYANGIITIS

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  • antineutrophil cytoplasm antibody-associated vasculitis
  • eosinophilic granulomatosis with polyangiitis
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