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

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 aimed 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-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 two-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 appendix 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.

Update in the Management of ANCA-Associated Vasculitis: Recent Developments and Future Perspectives

“…Hence, pulses of IV methylprednisolone may be considered in severe, organ or life-threatening GPA or MPA. However, “evidence is lacking to prove its efficacy and shows potential risk of adverse effects, mostly related to increased incidence of infections and diabetes,” as emphasized in the recently published CanVasc recommendations [ 5 ].…”

“…Because of the potential toxicity associated with CYC (cytopenias, infertility, and risk of hemorrhagic cystitis and bladder cancer), RTX has gained popularity for the induction of remission in AAV. Nevertheless, the long-term toxicity of CYC can be minimized by limiting its total lifetime cumulative dose to less 25 g [ 5 , 27 ].…”

“…RTX may be used as a first-line therapy for induction of remission with GC in severe GPA and MPA, especially in patients with contraindications to CYC [ 5 ]. Two main RCTs compared CYC and RTX head to head.…”

“…Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitides (AAV) are predominantly necrotizing, small vessel vasculitides and include granulomatosis with polyangiitis (GPA, previously known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) [ 1 , 2 ]. Serological markers include myeloperoxidase- (MPO-) ANCA and proteinase 3- (PR3-) ANCA, previously detected by immunofluorescence as perinuclear (p-ANCA) and cytoplasmic (c-ANCA), respectively [ 3 – 5 ].…”

Antineutrophil Cytoplasmic Antibody-associated Vasculitis Management 2020: Where Are We Now?

“…In parallel with these activities have been improvements in physician training and healthcare delivery, which have shortened diagnostic delay, and improved mortality and endstage renal disease risks 4 . Statements to guide AAV management have now been produced at national and international levels, and an update of the Canadian Vasculitis Society (CanVasc) 2015 statement is published in this edition of The Journal of Rheumatology 5 . This is an extensive piece of work with 39 recommendation statements, 15 of them new for this update covering diagnosis, drug therapies, eosinophilic granulomatosis with polyangiitis (EGPA), children, monitoring, and prophylaxis.…”