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Spring 2017 (Volume 27, Number 1)

CanVasc Recommendations for the Management of ANCA-associated Vasculitides

By Christian Pagnoux, MD, MSc, MPH; Nader A. Khalidi, MD, FRCP(C); and Lillian Barra, MD, PhD, FRCPC

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Vasculitides are potentially life-threatening diseases. Because of their relative rarity and heterogeneous clinical presentations, the management of individual patients can be extremely challenging.1 Treatment strategies may vary across different geographical regions depending on the specifics of healthcare system delivery, access to services and drug Treatments.2

The development of Canadian recommendations for the management of vasculitis is one of the means for the Canadian Vasculitis Network (CanVasc) to optimize and harmonize the care of patients with vasculitis in Canada and improve their outcomes. The first CanVasc recommendations, for the management of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV), were published in January 2016.3 Their dissemination is ongoing, and they are reaching larger audiences, including those abroad. Recently, Dr. Jack Cush picked them for a “news” vignette on RheumNow.com.4 The German Society of Rheumatology, which also recently developed its own recommendations (submitted for publication), included them in an article comparing similar initiatives, including the European League Against Rheumatism (EULAR)/ European Renal Association (ERA)-European Dialysis and Transplant Association (EDTA) updated recommendations, published in June 2016.5,6

When recommendations are being developed by different groups across the world for the same conditions, one resounding question is whether these similar initiatives are needed and useful or redundant and hence an unnecessary use of resources.

Of note, before these first CanVasc recommendations, no guidelines had ever been developed specifically for vasculitis in North America. Existing recommendations were exclusively from Europe, Japan and Australia.6-9 The international Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guidelines for management of glomerulonephritis included a section on pauci-immune glomerulonephritis, which is a frequent manifestation, but still only one aspect of AAV, which can affect many organ systems.10

Overall, the CanVasc and EULAR/ERA-EDTA recommendations are similar, which is reassuring. However, subtle differences are worth noting. There are specific Canadian healthcare issues which are addressed in the CanVasc recommendations. The CanVasc recommendations include statements on pregnancy and pediatric issues, not covered by the EULAR/ERA-EDTA guidelines. On the other hand, the latter mention mycophenolate mofetil (MMF) as an alternative to methotrexate, for the treatment of non-severe granulomatosis with polyangiitis (GPA). Available data were lacking for CanVasc to support such a recommendation. Unpublished results from a single randomized European study comparing MMF and cyclophosphamide are of limited convincing value.

Based on the recently published Maintenance of Remission in ANCA-vasculitis (MAINRITSAN) study,11 CanVasc mentioned rituximab as an "alternative to azathioprine for remission maintenance of [AAV], especially for patients with Proteinase 3 (PR3)-ANCA+ GPA."3 The EULAR/ERA-EDTA group listed azathioprine, methotrexate, rituximab and MMF as options for maintenance treatments.6 Rituximab is not yet approved for maintenance therapy in Canada and coverage varies across providers and provinces. The results of another ongoing international trial (RITAZAREM, ClinicalTrials.gov NCT01697267; last inclusion in November 2016) may confirm the role of rituximab for maintenance, but will not be available before late 2018.

The fact that more studies have been conducted and several recommendations developed over the past decade are good signs for patients with vasculitis, their physicians, and researchers. Those achievements may help vasculitis gain a more visible place in the Canadian rheumatology landscape, especially related to provincial payers and research funding agencies.

As new therapeutic options are currently under investigation, an update of the CanVasc recommendations will be needed when major developments occur. Until then, our medical community needs to be aware of and use the CanVasc recommendations for guidance in the management of patients with AAV. Several tools are under development to further promote their use and inclusion in the training and continuous learning of physicians managing vasculitis patients, including the forthcoming book, Canadian Vasculitis Learning Initiative (CaVALI): An Approach to Vasculitis Through Interactive Clinical Cases. For more information, please visit www.canvasc.ca.

References

1. Baldwin C, Carette S, Pagnoux C. Linking classification and therapeutic management of vasculitides. Arthritis Res Ther 2015; 17:138.

2. Famorca L, Twilt M, Barra L, et al. Development of Canadian recommendations for the management of ANCA-associated vasculitides: Results of the national needs assessment questionnaire. Open Rheumatol J 2015; 9:16-20.

3. McGeoch L, Twilt M, Famorca L, et al. CanVasc recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitides. J Rheumatol 2016; 43:97-120.

4. CanVasc Recommendations for ANCA-Associated Vasculitis. 2016. Accessed 19/01/2017, at http://rheumnow.com/content/canvasc-recommendations-anca-associated-vasculitis.

5. Csernok E, Kempiners N, Hellmich B. Paradigm shift in ANCA diagnostics: new international consensus recommendations. Z Rheumatol 2017 [Epub ahead of print].

6. Yates M, Watts RA, Bajema IM, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016 [Epub ahead of print].

7. Guerry MJ, Brogan P, Bruce IN, et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford) 2011; 51:634-43.

8. Guideline for management of vasculitis syndrome (JCS 2008). Japanese Circulation Society. Circ J 2008;75:474-503.

9. Menahem S, Hiremagalur B, Mudge D, et al. The CARI guidelines. Induction and maintenance therapy in ANCA-associated systemic vasculitis. Nephrology (Carlton) 2008;13 Suppl 2:S24-36.

10.Kidney Disease: Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group. KDIGO Clinical practice guideline for glomerulonephritis. Kidney inter 2012:139-274.

11.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.



Christian Pagnoux, MD, MSc, MPH
Vasculitis Clinic,
Division of Rheumatology,
Mount Sinai Hospital
Toronto, Ontario

Nader Khalidi, MD, FRCP(C)
Division of Rheumatology,
McMaster University
Hamilton, Ontario

Lillian Barra, MD, PhD, FRCPC
Division of Rheumatology,
St. Joseph's Health Care
University of Western Ontario,
London, Ontario

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