American College of Rheumatology. Vasculitis Guidelines. 2021. Accessed February 11, 2021. https://www.rheumatology.org/Practice-Quality/Clinical-Support/Clinical-Practice-Guidelines/Vasculitis
Basu N, McClean A, Harper L, et al. Markers for work disability in anti-neutrophil cytoplasmic antibody-associated vasculitis. Rheumatology (Oxford). 2014;53:953-6.
Basu N, McClean A, Harper L, et al. The characterisation and determinants of quality of life in ANCA associated vasculitis. Ann Rheum Dis. 2014;73:207-11.
Berti A, Matteson EL, Crowson CS, et al. Risk of cardiovascular disease and venous thromboembolism among patients with incident ANCA-associated vasculitis: a 20-year population-based cohort study. Mayo Clin Proc. 2018;93:597-606.
Chanouzas D, McGregor JAG, Nightingale P, et al. Intravenous pulse methylprednisolone for induction of remission in severe ANCA associated vasculitis: a multi-center retrospective cohort study. BMC Nephrol. 2019;20:58.
Charles P, Guillevin L. Long-term rituximab use to maintain remission of antineutrophil cytoplasmic antibody-associated vasculitis. Ann Intern Med. 2020;173:948.
Charles P, Terrier B, Perrodeau É, et al. Comparison of individually tailored versus fixed-schedule rituximab regimen to maintain ANCA-associated vasculitis remission: results of a multicentre, randomised controlled, phase III trial (MAINRITSAN2). Ann Rheum Dis. 2018;77:1143-1149. Erratum in: Ann Rheum Dis. 2019;78:e101.
ClinicalTrials.gov. A phase 3 clinical trial of CCX168 (avacopan) in patients with ANCA-associated vasculitis (ADVOCATE). Updated July 22, 2020. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT02994927
ClinicalTrials.gov. Efficacy and safety of rituximab in the treatment of good prognosis microscopic polyangiitis (RITUXGOPRO). Updated April 19, 2019. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT03920722
ClinicalTrials.gov. Evaluate the remission maintenance using extended administration of prednisone in systemic anti-neutrophil cytoplasmic antibodies (ANCA)-Associated Vasculitis. (MAINEPSAN). Updated November 22, 2019. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT03290456
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ClinicalTrials.gov. Rituximab and belimumab combination therapy in PR3 Vasculitis (COMBIVAS). Updated July 9, 2020. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT03967925
ClinicalTrials.gov. Study of IFX-1 to replace steroids in patients with granulomatosis with polyangiitis and microscopic polyangiitis. (IXchange). Updated January 12, 2021. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT03895801
ClinicalTrials.gov. The assessment of prednisone in remission trial - patient centric approach (TAPIR). Updated December 14, 2020. Accessed January 26, 2021. https://clinicaltrials.gov/ct2/show/NCT01933724
de Groot K, Harper L, Jayne DR, et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med. 2009;150:670-80.
Fauci AS, Katz P, Haynes BF, et al. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med. 1979;301:235-8.
Faurschou M, Westman K, Rasmussen N, et al. Brief Report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2012;64:3472-7.
Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70:488-94.
Guillevin L, Pagnoux C, Karras A, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771-80.
Harper L, Morgan MD, Walsh M, et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis. 2012;71:955-60.
Herlyn K, Hellmich B, Seo P, et al. Patient-reported outcome assessment in vasculitis may provide important data and a unique perspective. Arthritis Care Res (Hoboken). 2010;62:1639-45.
Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA. 2010;304:2381-8.
Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med. 2003;349:36-44.
Jayne DR, Chapel H, Adu D, et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM. 2000;93:433-9.
Jayne DRW, Bruchfeld AN, Harper L, et al. Randomized trial of C5a receptor inhibitor avacopan in ANCA-associated vasculitis. J Am Soc Nephrol. 2017;28:2756-2767.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65:1-11.
Jones RB, Hiemstra TF, Ballarin J, et al. Mycophenolate mofetil versus cyclophosphamide for remission induction in ANCA-associated vasculitis: a randomised, non-inferiority trial. Ann Rheum Dis.2019;78:399-405.
Jones RB, Tervaert JW, Hauser T, et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med. 2010;363:211-20.
Kallenberg CG, Heeringa P. Complement is crucial in the pathogenesis of ANCA-associated vasculitis. Kidney Int. 2013;83:16-8.
Kang A, Antonelou M, Wong NL, et al. High incidence of arterial and venous thrombosis in antineutrophil cytoplasmic antibody-associated vasculitis. J Rheumatol. 2019;46:285-293.
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Koutantji M, Harrold E, Lane SE, et al. Investigation of quality of life, mood, pain, disability, and disease status in primary systemic vasculitis. Arthritis Rheum. 2003;49:826-37.
Kronbichler A, Kerschbaum J, Gopaluni S, et al. Trimethoprim-sulfamethoxazole prophylaxis prevents severe/life-threatening infections following rituximab in antineutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2018;77:1440-1447.
Kronbichler A, Leierer J, Gauckler P, et al. Comorbidities in ANCA-associated vasculitis. Rheumatology (Oxford). 2020;59:iii79-iii83.
McAdoo SP, Medjeral-Thomas N, Gopaluni S, et al. Long-term follow-up of a combined rituximab and cyclophosphamide regimen in renal anti-neutrophil cytoplasm antibody-associated vasculitis. Nephrol Dial Transplant. 2019;34:63-73. Erratum in: Nephrol Dial Transplant. 2018;33:899.
Merkel PA, Jayne D, Yue H, et al. A randomized, double-blind, activecontrolled study of avacopan in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Ann Rheum Dis. 2020; doi:10.1136/annrheumdis-2020-eular.107A [Epub ahead of print]
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Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359:2790-803.
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Faculty

Neil Basu, MD, PhD

Clinical Senior Lecturer in Rheumatology
Honorary Consultant (Immunology)
Associate, Institute of Neuroscience & Psychology
University of Glasgow
Glasgow, United Kingdom

Disclosures

Disclosure: Neil Basu, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Gilead; GlaxoSmithKline; Lilly; Merck Sharp & Dohme, Corp.
Served as a speaker or a member of a speakers bureau for: AbbVie; Lilly; Roche; Vifor
Received grants for clinical research from: GlaxoSmithKline; Lilly; Pfizer; Vifor; Vorso

Joanna C Robson, MD, PhD, FRCP

Consultant Senior Lecturer in Rheumatology
Centre for Health and Clinical Research
University of the West of England, Bristol
Consultant Rheumatologist
University Hospitals Bristol and Weston NHS Foundation Trust
Bristol, United Kingdom

Disclosures

Disclosure: Joanna C. Robson, MD, PhD, FRCP, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Vifor
Received grants for clinical research from: Vifor

David Jayne, MD, FMedSci

Professor of Clinical Autoimmunity
University of Cambridge
Cambridge, United Kingdom

Disclosures

Disclosure: David Jayne, MD, FMedSci, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: AstraZeneca; Aurinia; Bristol Myers Squibb Company; Boehringer Ingelheim; Chemocentryx; Chugai; CSL Behring; Genentech; GlaxoSmithKline; Infla-RX; Janssen; Novartis; Roche; Takeda; Vifor
Received grants for clinical research from: GlaxoSmithKline
Owns stock, stock options, or bonds from: Aurinia

Y.K.O. Teng, MD, PhD

Department of Internal Medicine, Nephrology
Expert Center for Lupus -- Vasculitis- and Complement-Mediated
Systemic Autoimmune Diseases
Leiden University Medical Center
Leiden, the Netherlands

Disclosures

Disclosure: Y.K.O Teng, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Aurinia; GlaxoSmithKline; Novartis; Vifor
Received grants for clinical research from: Aurinia; GlaxoSmithKline; Vifor

Editors

George Boutsalis, PhD

Senior Medical Education Director, Medscape, LLC

Disclosures

Disclosure: George Boutsalis, PhD, has disclosed no relevant financial relationships.

Asha P. Gupta, PharmD, RPh

Senior Scientific Content Manager, Medscape, LLC

Disclosures

Disclosure: Asha P. Gupta, PharmD, RPh, has disclosed no relevant financial relationships.

Content Reviewer

Robert Morris, PharmD

Associate Director, Accreditation and Compliance

Disclosures

Disclosure: Robert Morris, PharmD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: ViiV Healthcare
Owns stock, stock options, or bonds from: GlaxoSmithKline

Medscape, LLC staff have disclosed that they have no relevant financial relationships.

Peer Reviewer

This activity has been peer reviewed and the reviewer has disclosed no relevant financial relationships.

CPD

ANCA-Associated Vasculitis: How Effective Is the Current Treatment Paradigm?

Authors: Neil Basu, MD, PhD; Joanna C. Robson, MD, PhD, FRCP; David Jayne, MD, FMedSci; Y.K.O. Teng, MD, PhD
CPD Released: 3/22/2021
THIS ACTIVITY HAS EXPIRED
Valid for credit through: 3/22/2022

Start Activity

Target Audience and Goal Statement

This educational activity is intended for an international audience of non-US nephrologists, rheumatologists, and primary care physicians.

The goal of this activity is to increase knowledge about current treatment options and their limitations, guideline recommendations, and recent clinical trial data for emerging therapies for patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV).

Upon completion of this activity, participants will:

Have increased knowledge regarding the
- Results from clinical trials that have compared the efficacy of immunosuppressive therapies used for remission-induction in patients with AAV
- Efficacy and safety of the C5a-targeted therapies in patients with AAV
- Guideline-based selection of the optimal duration of remission-maintenance therapy in patients with AAV

Disclosures

Faculty

Neil Basu, MD, PhD

Clinical Senior Lecturer in Rheumatology
Honorary Consultant (Immunology)
Associate, Institute of Neuroscience & Psychology
University of Glasgow
Glasgow, United Kingdom

Disclosures

Disclosure: Neil Basu, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Gilead; GlaxoSmithKline; Lilly; Merck Sharp & Dohme, Corp.
Served as a speaker or a member of a speakers bureau for: AbbVie; Lilly; Roche; Vifor
Received grants for clinical research from: GlaxoSmithKline; Lilly; Pfizer; Vifor; Vorso

Joanna C Robson, MD, PhD, FRCP

Consultant Senior Lecturer in Rheumatology
Centre for Health and Clinical Research
University of the West of England, Bristol
Consultant Rheumatologist
University Hospitals Bristol and Weston NHS Foundation Trust
Bristol, United Kingdom

Disclosures

Disclosure: Joanna C. Robson, MD, PhD, FRCP, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Vifor
Received grants for clinical research from: Vifor

David Jayne, MD, FMedSci

Professor of Clinical Autoimmunity
University of Cambridge
Cambridge, United Kingdom

Disclosures

Disclosure: David Jayne, MD, FMedSci, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: AstraZeneca; Aurinia; Bristol Myers Squibb Company; Boehringer Ingelheim; Chemocentryx; Chugai; CSL Behring; Genentech; GlaxoSmithKline; Infla-RX; Janssen; Novartis; Roche; Takeda; Vifor
Received grants for clinical research from: GlaxoSmithKline
Owns stock, stock options, or bonds from: Aurinia

Y.K.O. Teng, MD, PhD

Department of Internal Medicine, Nephrology
Expert Center for Lupus -- Vasculitis- and Complement-Mediated
Systemic Autoimmune Diseases
Leiden University Medical Center
Leiden, the Netherlands

Disclosures

Disclosure: Y.K.O Teng, MD, PhD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: Aurinia; GlaxoSmithKline; Novartis; Vifor
Received grants for clinical research from: Aurinia; GlaxoSmithKline; Vifor

Editors

George Boutsalis, PhD

Senior Medical Education Director, Medscape, LLC

Disclosures

Disclosure: George Boutsalis, PhD, has disclosed no relevant financial relationships.

Asha P. Gupta, PharmD, RPh

Senior Scientific Content Manager, Medscape, LLC

Disclosures

Disclosure: Asha P. Gupta, PharmD, RPh, has disclosed no relevant financial relationships.

Content Reviewer

Robert Morris, PharmD

Associate Director, Accreditation and Compliance

Disclosures

Disclosure: Robert Morris, PharmD, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: ViiV Healthcare
Owns stock, stock options, or bonds from: GlaxoSmithKline

Medscape, LLC staff have disclosed that they have no relevant financial relationships.

Peer Reviewer

This activity has been peer reviewed and the reviewer has disclosed no relevant financial relationships.

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For Physicians

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From Medscape Education

CPD

ANCA-Associated Vasculitis: How Effective Is the Current Treatment Paradigm?

Authors: Neil Basu, MD, PhD; Joanna C. Robson, MD, PhD, FRCP; David Jayne, MD, FMedSci; Y.K.O. Teng, MD, PhDFaculty and Disclosures

THIS ACTIVITY HAS EXPIRED

CPD Released: 3/22/2021

Valid for credit through: 3/22/2022

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Contents of This CPD Activity

All sections of this activity are required for credit.

Current Treatment Landscape for AAV

Neil Basu, MD, PhD

Challenges and Limitations of Current Treatment Practices

Joanna C. Robson, MD, PhD, FRCP

Guideline Recommendations for the Treatment of AAV

David Jayne, MD, FMedSci

Emerging Therapies in Development for AAV

Y.K.O. Teng, MD, PhD

Educational Impact Challenge

Before you begin this activity, please assess your clinical knowledge by completing this brief survey. Answering these questions again after the activity will allow you to see what you learned and to compare your answers with those of your peers.

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The educational activity presented above may involve simulated case-based scenarios. The patients depicted in these scenarios are fictitious and no association with any actual patient is intended or should be inferred.

The material presented here does not necessarily reflect the views of WebMD Global, LLC or companies that support educational programming on medscape.org. A qualified healthcare professional should be consulted before using any therapeutic product discussed. Readers should verify all information and data before treating patients or employing any therapies described in this educational activity.

References

Faculty and Disclosures

Faculty

Neil Basu, MD, PhD

Joanna C Robson, MD, PhD, FRCP

David Jayne, MD, FMedSci

Y.K.O. Teng, MD, PhD

Editors

George Boutsalis, PhD

Asha P. Gupta, PharmD, RPh

Content Reviewer

Robert Morris, PharmD

Peer Reviewer

CPD

ANCA-Associated Vasculitis: How Effective Is the Current Treatment Paradigm?

Target Audience and Goal Statement

Disclosures

Faculty

Neil Basu, MD, PhD

Joanna C Robson, MD, PhD, FRCP

David Jayne, MD, FMedSci

Y.K.O. Teng, MD, PhD

Editors

George Boutsalis, PhD

Asha P. Gupta, PharmD, RPh

Content Reviewer

Robert Morris, PharmD

Peer Reviewer

Accreditation Statements

For Physicians

Instructions for Participation and Credit

ANCA-Associated Vasculitis: How Effective Is the Current Treatment Paradigm?

Contents of This CPD Activity

Current Treatment Landscape for AAV

Challenges and Limitations of Current Treatment Practices

Guideline Recommendations for the Treatment of AAV

Emerging Therapies in Development for AAV

Educational Impact Challenge